BAG3

Summary

BAG3 (BAG cochaperone 3, HGNC:939) is a protein-coding gene on chromosome 10q26.11, encoding BAG family molecular chaperone regulator 3 (O95817). Co-chaperone and adapter protein that connects different classes of molecular chaperones including heat shock proteins 70 (HSP70s), e.g. It is haploinsufficient (ClinGen: sufficient evidence).

BAG proteins compete with Hip for binding to the Hsc70/Hsp70 ATPase domain and promote substrate release. All the BAG proteins have an approximately 45-amino acid BAG domain near the C terminus but differ markedly in their N-terminal regions. The protein encoded by this gene contains a WW domain in the N-terminal region and a BAG domain in the C-terminal region. The BAG domains of BAG1, BAG2, and BAG3 interact specifically with the Hsc70 ATPase domain in vitro and in mammalian cells. All 3 proteins bind with high affinity to the ATPase domain of Hsc70 and inhibit its chaperone activity in a Hip-repressible manner.

Source: NCBI Gene 9531 — RefSeq curated summary.

At a glance

• Gene–disease (curated): dilated cardiomyopathy 1HH (Definitive, ClinGen) — +6 more curated relationships
• GWAS associations: 34
• Clinical variants (ClinVar): 1,403 total — 108 pathogenic, 31 likely-pathogenic
• Phenotypes (HPO): 96
• Druggable target: yes
• Dosage sensitivity (ClinGen): haploinsufficiency sufficient evidence, triplosensitivity no evidence
• MANE Select transcript: NM_004281

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:939
Approved symbol	BAG3
Name	BAG cochaperone 3
Location	10q26.11
Locus type	gene with protein product
Status	Approved
Ensembl gene	ENSG00000151929
Ensembl biotype	protein_coding
OMIM	603883
Entrez	9531

Gene structure

Transcript identifiers

Ensembl transcripts: 8 — 8 protein_coding

ENST00000369085, ENST00000450186, ENST00000889977, ENST00000889978, ENST00000889979, ENST00000889980, ENST00000889981, ENST00000951434

RefSeq mRNA: 1 — MANE Select: NM_004281 NM_004281

CCDS: CCDS7615

Canonical transcript exons

ENST00000369085 — 4 exons

Exon	Start	End
ENSE00001002489	119669851	119670177
ENSE00001002492	119672255	119672656
ENSE00001448778	119676464	119677819
ENSE00001448780	119651380	119651855

Expression profiles

Bgee: expression breadth ubiquitous, 286 present calls, max score 99.09.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 75.0370 / max 1251.6871, expressed in 1804 samples.

FANTOM5 promoters (7 alternative TSS)

Promoter ID	TPM avg	Samples expressed
107324	37.6222	1787
107330	19.8686	1541
107325	10.8248	1719
107326	2.6667	1416
107329	1.9228	980
107327	1.0705	357
107328	1.0614	586

Top tissues by expression

288 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
gastrocnemius	UBERON:0001388	99.09	gold quality
skeletal muscle tissue of rectus abdominis	UBERON:0004511	98.98	gold quality
body of tongue	UBERON:0011876	98.97	gold quality
gluteal muscle	UBERON:0002000	98.91	gold quality
hindlimb stylopod muscle	UBERON:0004252	98.89	gold quality
quadriceps femoris	UBERON:0001377	98.67	gold quality
vastus lateralis	UBERON:0001379	98.66	gold quality
tibialis anterior	UBERON:0001385	98.61	gold quality
triceps brachii	UBERON:0001509	98.58	gold quality
muscle organ	UBERON:0001630	98.55	gold quality
muscle of leg	UBERON:0001383	98.54	gold quality
vena cava	UBERON:0004087	98.52	gold quality
skeletal muscle tissue	UBERON:0001134	98.48	gold quality
diaphragm	UBERON:0001103	98.38	gold quality
skeletal muscle tissue of biceps brachii	UBERON:0004502	98.36	gold quality
biceps brachii	UBERON:0001507	98.21	gold quality
deltoid	UBERON:0001476	98.11	gold quality
pharyngeal mucosa	UBERON:0000355	97.99	gold quality
muscle tissue	UBERON:0002385	97.83	gold quality
trachea	UBERON:0003126	97.63	gold quality
cardiac ventricle	UBERON:0002082	97.31	gold quality
heart left ventricle	UBERON:0002084	97.31	gold quality
tongue	UBERON:0001723	97.24	gold quality
dorsal root ganglion	UBERON:0000044	97.21	gold quality
cardiac atrium	UBERON:0002081	97.21	gold quality
right atrium auricular region	UBERON:0006631	97.20	gold quality
saphenous vein	UBERON:0007318	97.13	gold quality
nipple	UBERON:0002030	97.07	gold quality
heart right ventricle	UBERON:0002080	96.96	gold quality
apex of heart	UBERON:0002098	96.89	gold quality

Single-cell (SCXA)

Detected in 4 experiment(s), a significant marker in 2.

Experiment	Marker?	Max mean expression
E-GEOD-81547	yes	9.75
E-CURD-10	no	1027.00
E-MTAB-10137	no	9.98
E-ANND-3	no	0.00

Regulation

Is transcription factor: yes

Downstream targets (CollecTRI)

1 targets.

Target	Regulation
SIRT5	Repression

Upstream regulators (CollecTRI, top): CTNNB1, DNMT1, DNMT3B, EGR1, GTF3A, HSF1, IRF6, TFAP2A

miRNA regulators (miRDB)

39 targeting BAG3, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-656-3P	100.00	72.15	2788
HSA-MIR-126-5P	100.00	72.71	3180
HSA-MIR-7110-3P	100.00	73.18	2486
HSA-MIR-4795-3P	100.00	74.62	4024
HSA-MIR-196A-1-3P	99.99	72.15	2772
HSA-MIR-1250-3P	99.96	70.04	4038
HSA-MIR-6835-3P	99.93	70.49	2904
HSA-LET-7A-2-3P	99.87	70.53	1921
HSA-MIR-548D-3P	99.87	70.67	4362
HSA-MIR-221-5P	99.86	65.45	1052
HSA-MIR-8073	99.86	65.21	1118
HSA-MIR-548BB-3P	99.86	70.58	4354
HSA-LET-7G-3P	99.85	70.43	1929
HSA-MIR-548AC	99.84	70.77	4351
HSA-MIR-548H-3P	99.84	70.80	4349
HSA-MIR-548Z	99.84	70.80	4349
HSA-MIR-944	99.82	70.85	3042
HSA-MIR-646	99.68	67.84	1645
HSA-MIR-891B	99.59	69.81	1083
HSA-MIR-6832-3P	99.52	70.44	1726
HSA-MIR-143-3P	99.49	69.05	1457
HSA-MIR-4770	99.49	69.09	1451
HSA-MIR-217-5P	99.49	69.93	1419
HSA-MIR-12132	99.47	68.90	1341
HSA-MIR-4762-3P	99.43	69.72	2363
HSA-MIR-4797-5P	99.39	68.01	1354
HSA-MIR-6507-5P	99.36	70.46	2524
HSA-MIR-6507-3P	99.35	67.32	1059
HSA-MIR-520F-5P	99.34	70.40	1632
HSA-MIR-6088	99.29	68.45	1284

Functional genomics

ClinGen dosage: haploinsufficiency 3 (sufficient evidence), triplosensitivity 0 (no evidence). ClinGen Gene Dosage Map

Literature-anchored findings (GeneRIF, showing 40)

• BAG3 protein has a role in apoptosis in B-chronic lymphocytic leukaemia cells (PMID:12700638)
• heavy metals and temperature regulate BAG3 gene expression. (PMID:12706811)
• findings suggest that heat shock protein-70 is a chaperone driving a multiprotein degradation complex and that the inhibitory co-chaperone CAIR-1 protein functions distal to client ubiquitination (PMID:12750378)
• These results suggest that the Bis induced growth inhibition of HL-60 cells promotes G0/G1 phase arrest via up-regulation of p27, which seems to be a prerequisite for differentiation. (PMID:16391524)
• Results indicate that CAIR-1 may negatively regulate adhesion, focal adhesion assembly, signaling, and migration via its PXXP domain. (PMID:16859681)
• BAG3 downmodulates the apoptotic response to TRAIL in human neoplastic thyroid cells. (PMID:17164298)
• These observations reveal a previously unrecognized cell response, that is, an increase in BAG3, elicited by HIV-1 infection, and may provide a new avenue for the suppression of HIV-1 gene expression. (PMID:17187345)
• The metabolic pathway of BAG3 was studied. (PMID:17507989)
• High levels of BAG3 protein seen in some epithelial cancer cell lines may be relevant to mechanisms of tumor invasion and metastasis. (PMID:17974966)
• Taken together, our results suggest that BAG3 induction might represents as an unwanted molecular consequence of utilizing proteasome inhibitors to combat tumors. (PMID:17996194)
• These results suggested that the HspB8-Bag3 complex might stimulate the degradation of Htt43Q by macroautophagy. (PMID:18006506)
• HspB8 and Bag3: a new chaperone complex targeting misfolded proteins to macroautophagy. (PMID:18094623)
• Two of the three putative heat shock-responsive elements (HSEs) in bag3 promoter interact with the heat shock factor (HSF) 1 in vitro and in vivo. (PMID:18286539)
• Activation of BAG3 by Egr-1 in response to FGF-2 in neuroblastoma. (PMID:18469860)
• Downmodulation of BAG3 protein levels allows caspase-3 activation by HIV-1 infection in human primary microglial cells. (PMID:18821563)
• This study conclude mutation in Bag3 defines a novel severe autosomal dominant childhood muscular dystrophy. (PMID:19085932)
• a requirement for BAG3 to augment virus gene expression and demonstrate that the co-chaperone acts independently of promyelocytic leukemia to increase herpes simplex virus replication. (PMID:19088197)
• These results indicate that knocking down BAG3 gene is a promising new approach to enhance the therapeutic potency of Bortezomib in leukemia. (PMID:19111544)
• in a B-cell lymphoma cell line, physical interaction was observed between BTK and BAG3, possibly upon modifications induced by stress (PMID:19212330)
• This study showed that BAG3 protein is downregulated upon JC virus (JCV) infection and that this effect is mediated by JCV T-antigen via repression of the BAG3 promoter. (PMID:19282432)
• Loss of BAG3 under STS injury required sequential caspase cleavage followed by polyubiquitination and proteasomal degradation (PMID:19352495)
• BAG3 gene expression is controlled by its own product. (PMID:19777443)
• Data show that the interaction between HspB6 and Bag3 requires the same regions that are involved in the HspB8-Bag3 association. (PMID:19845507)
• identification of BAG3 binding partner (cytosolic chaperonin CCT); BAG3/CTT complex binds to BAG3 promoter & exhibits biological function (i.e., influences correct folding of monomeric actin); study of BAG3 domain participation in binding to CCT (PMID:20018251)
• BAG3 is cleaved during apoptosis of pancreatic cancer cells, disrupting its anti-apoptotic properties. (PMID:20232307)
• BAG3 alters the interaction between HSP70 and IKKgamma, increasing availability of IKKgamma and protecting it from proteasome-dependent degradation; this, in turn, results in increased NF-kappaB activity and survival (PMID:20368414)
• BAG3 intra-cytoplasmic delocalisation is a specific feature of cancer versus non-neoplastic prostate and a candidate new marker for prediction of prostate cancer invasiveness and behaviour. (PMID:20535599)
• These observations suggest that the BAG3 variant of myofibrillar myopathy may result from a spontaneous mutation at an early point of embryonic development and that transmission from a mosaic parent may occur more than once. (PMID:20605452)
• interaction between virus penton base protein and cellular co-chaperone BAG3 positively influences virus life cycle (PMID:20607728)
• bag3 expression in chronic lymphocytic leukemia patients is markedly higher than that in normal controls, while high bag3 level in CML patients is probably related with drug resistance, but is not related with clinically established prognostic factors. (PMID:20723284)
• BAG3 overexpression increases cell adhesion in Cos7 cells, but not in PDZGEF2 gene knockdown cells indicating that PDZGEF2 is a critical partner for BAG3 in regulating cell adhesion (PMID:20800573)
• BAG3 expression in glioblastoma cells promotes accumulation of ubiquitinated clients in an Hsp70-dependent manner. (PMID:21233200)
• aggresome-targeting by BAG3 is distinct from previously described mechanisms, as it does not depend on substrate ubiquitination (PMID:21252941)
• BAG3 was identified as a marker of electromagnetic field-induced stress. These results suggest that BAG3 induction by an electromagnetic field may contribute to melanoma cell survival and/or resistance to therapy. (PMID:21302292)
• The incidence of BAG3 positivity was significantly higher at advanced clinical stages of ovarian cancer than at early stages. It is suggected BAG3 binds to MMP2 to positively regulate the process of cell invasion. (PMID:21316839)
• New comprehensive genomic approaches have identified rare variants in BAG3 as causative of dilated cardiomyopathy. (PMID:21353195)
• the first Chinese case of Bag3opathy so far reported. (PMID:21361913)
• These findings indicate a novel function for BAG3 in inhibiting protein aggregation caused by the genetic mutation of CRYAB responsible for human myofibrillar myopathy. (PMID:21423662)
• BAG3 promotes the emergence of castration resistant prostate cancer by modulating IKKalpha nuclear translocation. (PMID:21451574)
• The results show that rare mutations in BAG3 contribute to monogenic forms of the disease, while common variant(s) in the same gene are implicated in sporadic DCM (PMID:21459883)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	bag3	ENSDARG00000039486
mus_musculus	Bag3	ENSMUSG00000030847
rattus_norvegicus	Bag3	ENSRNOG00000020298
drosophila_melanogaster	stv	FBGN0086708

Paralogs (2): BAG4 (ENSG00000156735), BAG5 (ENSG00000166170)

Protein

Protein identifiers

BAG family molecular chaperone regulator 3 — O95817 (reviewed: O95817)

Alternative names: Bcl-2-associated athanogene 3, Bcl-2-binding protein Bis, Docking protein CAIR-1

All UniProt accessions (3): O95817, A0A872G2N3, C9JFK9

UniProt curated annotations — full annotation on UniProt →

Function. Co-chaperone and adapter protein that connects different classes of molecular chaperones including heat shock proteins 70 (HSP70s), e.g. HSPA1A/HSP70 or HSPA8/HSC70, and small heat shock proteins (sHSPs), e.g. HSPB8. Acts as a nucleotide-exchange factor (NEF) promoting the release of ADP from HSP70s, thereby triggering client protein release. Nucleotide release is mediated via BAG3 binding to the nucleotide-binding domain (NBD) of HSP70s, whereas client release is mediated via binding to the substrate-binding domain (SBD). Has anti-apoptotic activity. Plays a role in the HSF1 nucleocytoplasmic transport.

Subunit / interactions. Forms a ternary complex with HSPA1A/HSP70 and HSPB8, serving as scaffold subunit. Component of the chaperone-assisted selective autophagy (CASA) complex consisting of BAG3, HSPA8/HSC70, HSPB8 and STUB1/CHIP. Binds to the ATPase domain of HSP70 chaperones. Interacts with BCL2. Interacts with phospholipase C-gamma proteins. Interacts with DNAJB1 and DNAJB6. Interacts (via BAG domain) with HSF1; this interaction occurs in normal and heat-shocked cells promoting HSF1 nucleocytoplasmic shuttling. Interacts with HSPA8/HSC70 (via NBD). Interacts with HSPA1A (via NBD) and HSPA1B (via NBD). Interacts (via WW domain 1) with SYNPO2 (via PPPY motif). Interacts with HSPB8.

Subcellular location. Nucleus. Cytoplasm.

Disease relevance. Myopathy, myofibrillar, 6 (MFM6) [MIM:612954] A form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disk and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFM6 is characterized by early-onset of severe, progressive, diffuse muscle weakness associated with cardiomyopathy, severe respiratory insufficiency during adolescence, and a rigid spine in some patients. The disease is caused by variants affecting the gene represented in this entry. Cardiomyopathy, dilated, 1HH (CMD1HH) [MIM:613881] A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. The disease is caused by variants affecting the gene represented in this entry. Neuronopathy, distal hereditary motor, autosomal dominant 15 (HMND15) [MIM:621094] A form of distal hereditary motor neuronopathy, a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. Weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. HMND15 is an adult-onset, autosomal dominant form with incomplete penetrance. HMND15 affected individuals exhibit a slowly progressive and symmetric distal weakness and atrophy of lower limb muscles, along with absent Achille reflexes. Sensory deficits are not present. The disease is caused by variants affecting the gene represented in this entry. Charcot-Marie-Tooth disease, axonal, type 2JJ (CMT2JJ) [MIM:621095] An autosomal dominant, axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2JJ is characterized by adult onset of distal sensory impairment and distal muscle weakness and atrophy predominantly affecting the lower limbs. The disease is caused by variants affecting the gene represented in this entry.

Domain organisation. The BAG domain interacts with the nucleotide-binding domain (NBD) of HSP70s and is essential for the nucleotide-exchange factor activity.

RefSeq proteins (1): NP_004272* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR001202	WW_dom	Domain
IPR003103	BAG_domain	Domain
IPR036020	WW_dom_sf	Homologous_superfamily
IPR036533	BAG_dom_sf	Homologous_superfamily
IPR039773	BAG_chaperone_regulator	Family

Pfam: PF00397, PF02179

UniProt features (63 total): sequence variant 22, modified residue 17, compositionally biased region 9, region of interest 4, domain 3, sequence conflict 3, cross-link 2, initiator methionine 1, chain 1, mutagenesis site 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-O95817-F1	57.98	0.19

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Post-translational modifications (19): 2, 136, 139, 173, 198, 261, 269, 274, 275, 279, 285, 289, 291, 377, 385, 386, 406, 445, 445

Mutagenesis-validated functional residues (1):

Position	Phenotype
480–481	significant loss of interaction with hspa8.

Function

Pathways and Gene Ontology

Reactome pathways

4 pathways

ID	Pathway
R-HSA-3371453	Regulation of HSF1-mediated heat shock response
R-HSA-2262752	Cellular responses to stress
R-HSA-3371556	Cellular response to heat stress
R-HSA-8953897	Cellular responses to stimuli

MSigDB gene sets: 512 (showing top): GOBP_SPINAL_CORD_DEVELOPMENT, GOBP_REGULATION_OF_AUTOPHAGY, GOBP_VACUOLE_ORGANIZATION, GOBP_INTRACELLULAR_PROTEIN_TRANSPORT, MODULE_255, GOBP_CELLULAR_RESPONSE_TO_EXTERNAL_STIMULUS, GOLDRATH_IMMUNE_MEMORY, GOBP_POSITIVE_REGULATION_OF_INTRACELLULAR_PROTEIN_TRANSPORT, GOZGIT_ESR1_TARGETS_DN, GOBP_POSITIVE_REGULATION_OF_PROTEIN_LOCALIZATION, GOBP_PROTEIN_TARGETING, GOBP_MACROMOLECULE_CATABOLIC_PROCESS, MODULE_317, GOBP_REGULATION_OF_PROTEIN_EXPORT_FROM_NUCLEUS, GOBP_EXTRINSIC_APOPTOTIC_SIGNALING_PATHWAY

GO Biological Process (22): autophagosome assembly (GO:0000045), protein folding (GO:0006457), extrinsic apoptotic signaling pathway via death domain receptors (GO:0008625), striated muscle cell apoptotic process (GO:0010658), negative regulation of striated muscle cell apoptotic process (GO:0010664), spinal cord development (GO:0021510), cellular response to heat (GO:0034605), cellular response to unfolded protein (GO:0034620), positive regulation of protein import into nucleus (GO:0042307), negative regulation of apoptotic process (GO:0043066), muscle cell cellular homeostasis (GO:0046716), positive regulation of protein export from nucleus (GO:0046827), protein stabilization (GO:0050821), chaperone-mediated autophagy (GO:0061684), aggresome assembly (GO:0070842), cellular response to mechanical stimulus (GO:0071260), extrinsic apoptotic signaling pathway in absence of ligand (GO:0097192), protein transport along microtubule (GO:0098840), obsolete negative regulation of protein targeting to mitochondrion (GO:1903215), positive regulation of aggrephagy (GO:1905337), apoptotic process (GO:0006915), extrinsic apoptotic signaling pathway (GO:0097191)

GO Molecular Function (8): adenyl-nucleotide exchange factor activity (GO:0000774), protein-macromolecule adaptor activity (GO:0030674), protein-containing complex binding (GO:0044877), cadherin binding (GO:0045296), dynein intermediate chain binding (GO:0045505), protein-folding chaperone binding (GO:0051087), protein carrier activity (GO:0140597), protein binding (GO:0005515)

GO Cellular Component (11): stress fiber (GO:0001725), nucleus (GO:0005634), nucleoplasm (GO:0005654), cytoplasm (GO:0005737), mitochondrion (GO:0005739), cytosol (GO:0005829), membrane (GO:0016020), aggresome (GO:0016235), Z disc (GO:0030018), ciliary basal body (GO:0036064), protein folding chaperone complex (GO:0101031)

Reactome top-level categories

Rollup of top-3 pathways:

Category	Pathways
Cellular response to heat stress	1
Cellular responses to stimuli	1
Cellular responses to stress	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	5
protein binding	3
extrinsic apoptotic signaling pathway	2
positive regulation of nucleocytoplasmic transport	2
positive regulation of intracellular protein transport	2
binding	2
intracellular membrane-bounded organelle	2
cytoplasm	2
Atg12 activating enzyme activity	1
protein-phosphatidylethanolamide deconjugating activity	1
Atg12 conjugating enzyme activity	1
Atg12 ligase activity	1
organelle assembly	1
Atg1/ULK1 kinase complex assembly	1
autophagosome organization	1
cellular process	1
protein maturation	1
muscle cell apoptotic process	1
negative regulation of muscle cell apoptotic process	1
striated muscle cell apoptotic process	1
regulation of striated muscle cell apoptotic process	1
central nervous system development	1
anatomical structure development	1
response to heat	1
cellular response to stress	1
response to unfolded protein	1
cellular response to topologically incorrect protein	1
protein import into nucleus	1
regulation of protein import into nucleus	1
positive regulation of protein localization to nucleus	1
apoptotic process	1
regulation of apoptotic process	1
negative regulation of programmed cell death	1
cellular homeostasis	1
protein export from nucleus	1
regulation of protein export from nucleus	1
regulation of protein stability	1
signaling receptor binding	1
autophagy	1
protein catabolic process	1

Protein interactions and networks

STRING

3592 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
BAG3	HSPB8	Q9UJY1	997
BAG3	HSPA4	P34932	997
BAG3	HSPA8	P11142	997
BAG3	STUB1	Q9UNE7	991
BAG3	BCL2	P10415	982
BAG3	BAG1	Q99933	935
BAG3	BAG2	O95816	934
BAG3	FLNC	Q14315	914
BAG3	MYOT	Q9UBF9	894
BAG3	HSPB1	P04792	882
BAG3	CRYAB	P02511	875
BAG3	DNAJB6	O75190	862
BAG3	HSP90AA1	P07900	846
BAG3	SQSTM1	Q13501	845
BAG3	HSPB6	O14558	827

IntAct

436 interactions, top by confidence:

A	B	Type	Score
BAG3	HSPB8	psi-mi:“MI:0915”(physical association)	0.930
PDLIM7	BAG3	psi-mi:“MI:0914”(association)	0.800
BAG3	PDLIM7	psi-mi:“MI:0915”(physical association)	0.800
DAZAP2	BAG3	psi-mi:“MI:0915”(physical association)	0.780
BAG3	LITAF	psi-mi:“MI:0915”(physical association)	0.780
LITAF	BAG3	psi-mi:“MI:0915”(physical association)	0.780
BAG3	DAZAP2	psi-mi:“MI:0915”(physical association)	0.780
SF3B4	BAG3	psi-mi:“MI:0915”(physical association)	0.720
ARRDC3	BAG3	psi-mi:“MI:0915”(physical association)	0.720
MAGED1	BAG3	psi-mi:“MI:0915”(physical association)	0.720
BAG3	ARRDC3	psi-mi:“MI:0915”(physical association)	0.720
BAG3	MAGED1	psi-mi:“MI:0915”(physical association)	0.720
BAG3	SF3B4	psi-mi:“MI:0915”(physical association)	0.720
BAG3	ATN1	psi-mi:“MI:0915”(physical association)	0.720
BAG3	hspa1a_hspa1b_human-1	psi-mi:“MI:0915”(physical association)	0.670
BAG3	HSPB2	psi-mi:“MI:0915”(physical association)	0.670
BAG3	WBP2	psi-mi:“MI:0915”(physical association)	0.670

BioGRID (929): BAG3 (Two-hybrid), BAG3 (Two-hybrid), BAG3 (Two-hybrid), BAG3 (Two-hybrid), DAZAP2 (Two-hybrid), SF3B4 (Two-hybrid), ARRDC3 (Two-hybrid), BAG3 (Affinity Capture-RNA), BAG3 (Affinity Capture-MS), BAG3 (Affinity Capture-MS), BAG3 (Affinity Capture-MS), BAG3 (Affinity Capture-MS), BAG3 (Affinity Capture-MS), BAG3 (Two-hybrid), BAG3 (Two-hybrid)

ESM2 similar proteins: A0A1L9WLD5, A2QUM3, A3LTE2, A7RNZ0, B0XXN8, B2ABT3, B6QFV5, B8M9Q9, C0NTV7, C7Z403, D1ZMX7, D4AZ68, E6R3N7, G4MVZ1, J9VPM2, J9VT33, M1VWN2, O00167, O54946, O95817, P02672, P0CS26, P0CS27, P36228, P81584, P91859, Q0CGY7, Q12329, Q1HVC7, Q27002, Q4P7Q1, Q4WXY0, Q4X212, Q58DB6, Q5JWF2, Q6AYU3, Q6BJD4, Q6C0K9, Q6CEV2, Q6CFG3

Diamond homologs: A0A8C0NGY6, A0A8I3PQN6, A1A5G4, A1CQG2, A1D3C5, A2QQ28, A4IIJ3, B0XQ72, B3LWS4, B3P3M8, B4HEJ6, B4K6I9, B4M5X4, B4NAD3, B4PSQ2, B8N7E5, D6C652, G0S9J5, H2LBU8, O14326, O88382, O95817, P39940, P46934, P46935, P46936, P46937, P46938, Q0CCL1, Q19404, Q1L8J7, Q2EJA0, Q2UBP1, Q32NJ6, Q4L1J4, Q4WTF3, Q5BDP1, Q5F488, Q5TCQ9, Q62940

SIGNOR signaling

11 interactions.

A	Effect	B	Mechanism
PRKCD	up-regulates	BAG3	phosphorylation
DNMT1	“up-regulates quantity by expression”	BAG3	“transcriptional regulation”
DNMT3B	“up-regulates quantity by expression”	BAG3	“transcriptional regulation”
BAG3	“down-regulates quantity by repression”	SIRT5	“transcriptional regulation”
ERK1/2	“up-regulates activity”	BAG3	phosphorylation
MAPK3	“down-regulates quantity by destabilization”	BAG3	phosphorylation
FBXO22	“down-regulates quantity by destabilization”	BAG3	binding
“Cullin 1-RBX1-Skp1”	“down-regulates quantity by destabilization”	BAG3	ubiquitination
CDK5	“down-regulates quantity by destabilization”	BAG3	phosphorylation
BAG3	“up-regulates activity”	HSPA8	binding

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 107 IntAct physical interaction partners (hypergeometric vs the genome-wide background, BH-FDR, gene-set size 15–500, ranked by fold). A functional readout of the neighbourhood — distinct from this gene’s own memberships above, and biased toward well-studied / hub proteins, so read it as themes rather than proof.

Reactome pathways:

Pathway	Partners	Fold	FDR
HSF1-dependent transactivation	7	36.4×	3e-07

Disease & clinical

Clinical variants and AI predictions

ClinVar

1403 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	108
Likely pathogenic	31
Uncertain significance	755
Likely benign	332
Benign	27

Top pathogenic / likely-pathogenic (30)

Variant ID	HGVS	Classification
1066195	NM_004281.4(BAG3):c.1348A>T (p.Lys450Ter)	Pathogenic
1069307	NM_004281.4(BAG3):c.811C>T (p.Gln271Ter)	Pathogenic
1070601	NM_004281.4(BAG3):c.824del (p.Ser275fs)	Pathogenic
1075312	NM_004281.4(BAG3):c.351_352insTGGATGCAGCGATTCCGAACTGAGGCGGCAGCAGCGGCTCCTCAGAGGTCCCAGTCACCTCTGCGGGGCATGCCAGAAACCACTCAGCCAGATAAACAGCGTGGACAGGTGGCAGCGGCGGCGGCAGCCCAGCCCCCAGCCT (p.Gly118fs)	Pathogenic
1075572	NM_004281.4(BAG3):c.530_531del (p.Ala176_Ser177insTer)	Pathogenic
1076610	NM_004281.4(BAG3):c.654del (p.Pro219fs)	Pathogenic
1076893	NC_000010.10:g.(?121411178)(121437012_?)del	Pathogenic
1188147	NM_004281.4(BAG3):c.709C>T (p.Gln237Ter)	Pathogenic
1324072	NM_004281.4(BAG3):c.457C>T (p.Gln153Ter)	Pathogenic
1324087	NM_004281.4(BAG3):c.331_332del (p.Phe111fs)	Pathogenic
1324125	NM_004281.4(BAG3):c.1057del (p.Gln353fs)	Pathogenic
1362749	NM_004281.4(BAG3):c.598C>T (p.Gln200Ter)	Pathogenic
1396666	NM_004281.4(BAG3):c.165del (p.Ser56fs)	Pathogenic
1398633	NM_004281.4(BAG3):c.640C>T (p.Gln214Ter)	Pathogenic
1452208	NM_004281.4(BAG3):c.38_39dup (p.Ser14fs)	Pathogenic
1453625	NM_004281.4(BAG3):c.488del (p.Pro163fs)	Pathogenic
1455927	NC_000010.10:g.(?121411188)(121411387_?)del	Pathogenic
1456226	NM_004281.4(BAG3):c.1326dup (p.Glu443Ter)	Pathogenic
1456480	NM_004281.4(BAG3):c.766G>T (p.Glu256Ter)	Pathogenic
1457623	NC_000010.10:g.(?121435956)(121436794_?)del	Pathogenic
1494973	NM_004281.4(BAG3):c.1088_1708del (p.Glu363_Pro569del)	Pathogenic
156530	NM_004281.4(BAG3):c.626C>A (p.Pro209Gln)	Pathogenic
1736414	NM_004281.4(BAG3):c.394C>T (p.Gln132Ter)	Pathogenic
1762103	NM_004281.4(BAG3):c.1257del (p.Pro420fs)	Pathogenic
1797440	NM_004281.4(BAG3):c.29_35del (p.Met10fs)	Pathogenic
1804144	NC_000010.11:g.119675031_119678711del	Pathogenic
1805113	NM_004281.4(BAG3):c.751dup (p.Gln251fs)	Pathogenic
1879146	NM_004281.4(BAG3):c.447dup (p.Gln150fs)	Pathogenic
1997860	NM_004281.4(BAG3):c.358C>T (p.Gln120Ter)	Pathogenic
2007830	NM_004281.4(BAG3):c.96_97del (p.Gln33fs)	Pathogenic

SpliceAI

730 predictions. Top by Δscore:

Variant	Effect	Δscore
10:119651855:GGTG:G	donor_loss	1.0000
10:119651856:GTGA:G	donor_loss	1.0000
10:119670178:G:GG	donor_gain	1.0000
10:119670179:T:G	donor_loss	1.0000
10:119675720:G:GG	donor_gain	1.0000
10:119676458:TTTCA:T	acceptor_loss	1.0000
10:119676459:TTCA:T	acceptor_loss	1.0000
10:119676460:TCAG:T	acceptor_loss	1.0000
10:119676461:CAG:C	acceptor_loss	1.0000
10:119676462:A:AG	acceptor_gain	1.0000
10:119676462:A:AT	acceptor_loss	1.0000
10:119676463:G:A	acceptor_loss	1.0000
10:119676463:G:GG	acceptor_gain	1.0000
10:119676463:GC:G	acceptor_gain	1.0000
10:119676463:GCA:G	acceptor_gain	1.0000
10:119676463:GCAGC:G	acceptor_gain	1.0000
10:119669849:AG:A	acceptor_gain	0.9900
10:119669850:GG:G	acceptor_gain	0.9900
10:119669850:GGA:G	acceptor_gain	0.9900
10:119670175:GAG:G	donor_gain	0.9900
10:119672253:AGC:A	acceptor_gain	0.9900
10:119672254:GCG:G	acceptor_gain	0.9900
10:119672461:G:T	donor_gain	0.9900
10:119675690:G:GT	donor_gain	0.9900
10:119675696:GCTC:G	donor_gain	0.9900
10:119675699:C:G	donor_gain	0.9900
10:119675717:GCA:G	donor_gain	0.9900
10:119675726:A:AG	donor_gain	0.9900
10:119676466:G:GA	acceptor_gain	0.9900
10:119676466:GC:G	acceptor_gain	0.9900

AlphaMissense

3711 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
10:119651751:T:A	W26R	1.000
10:119651751:T:C	W26R	1.000
10:119651753:G:C	W26C	1.000
10:119651753:G:T	W26C	1.000
10:119651820:T:A	W49R	1.000
10:119651820:T:C	W49R	1.000
10:119651822:G:C	W49C	1.000
10:119651822:G:T	W49C	1.000
10:119676879:T:C	F442S	1.000
10:119676905:T:C	Y451H	1.000
10:119676905:T:G	Y451D	1.000
10:119676909:T:C	L452P	1.000
10:119676919:A:C	E455D	1.000
10:119676919:A:T	E455D	1.000
10:119676927:T:C	L458S	1.000
10:119676927:T:G	L458W	1.000
10:119676939:T:C	L462P	1.000
10:119676942:T:A	L463Q	1.000
10:119676942:T:C	L463P	1.000
10:119676948:T:A	L465Q	1.000
10:119676948:T:C	L465P	1.000
10:119676950:G:C	D466H	1.000
10:119676951:A:C	D466A	1.000
10:119676951:A:T	D466V	1.000
10:119676953:T:C	S467P	1.000
10:119676993:G:C	R480T	1.000
10:119676993:G:T	R480M	1.000
10:119676994:G:C	R480S	1.000
10:119676994:G:T	R480S	1.000
10:119677002:G:A	G483D	1.000

dbSNP variants (sampled 300 via entrez): RS1000014560 (10:119650170 G>A), RS1000054175 (10:119654656 C>T), RS1000124937 (10:119667068 C>T), RS1000225409 (10:119660374 T>C,G), RS1000232601 (10:119652096 G>A), RS1000517056 (10:119663358 G>A), RS1000581055 (10:119651267 C>A,T), RS1000588844 (10:119664629 A>G), RS1000652314 (10:119652237 G>A,C), RS1000806683 (10:119668775 A>T), RS1000828268 (10:119667300 T>A), RS1000852378 (10:119675687 C>G,T), RS1000883310 (10:119661025 A>G), RS1000891646 (10:119678005 C>G,T), RS1001027993 (10:119656468 T>C)

Disease associations

OMIM: gene MIM:603883 | disease phenotypes: MIM:612954, MIM:613881, MIM:115200, MIM:192600, MIM:604169, MIM:621095, MIM:621094, MIM:107970, MIM:601419

GenCC curated gene-disease

Disease	Classification	Inheritance
myofibrillar myopathy	Definitive	Unknown
dilated cardiomyopathy 1HH	Definitive	Autosomal dominant
myofibrillar myopathy 6	Strong	Autosomal dominant
neuronopathy, distal hereditary motor, autosomal dominant	Moderate	Autosomal dominant
familial isolated dilated cardiomyopathy	Supportive	Autosomal dominant
distal hereditary motor neuropathy	Limited	Autosomal dominant
Charcot-Marie-tooth disease, axonal, type 2JJ	Limited	Autosomal dominant

ClinGen Gene-Disease Validity (2)

Expert-panel classifications — Definitive > Strong > Moderate > Limited > Disputed > Refuted.

Disease	Classification	Inheritance
dilated cardiomyopathy 1HH	Definitive	AD
myofibrillar myopathy	Definitive	AD

Mondo (20): myofibrillar myopathy 6 (MONDO:0013061), dilated cardiomyopathy 1HH (MONDO:0013479), cardiomyopathy (MONDO:0004994), familial dilated cardiomyopathy (MONDO:0016333), dilated cardiomyopathy (MONDO:0005021), atrial fibrillation (MONDO:0004981), long QT syndrome (MONDO:0002442), myocarditis (MONDO:0004496), familial hypertrophic cardiomyopathy (MONDO:0024573), left ventricular noncompaction 1 (MONDO:0011403), Charcot-Marie-tooth disease, axonal, type 2JJ (MONDO:0976227), neuronopathy, distal hereditary motor, autosomal dominant 15 (MONDO:0976226), familial isolated arrhythmogenic right ventricular dysplasia (MONDO:0016342), myofibrillar myopathy (MONDO:0018943), hypertrophic cardiomyopathy (MONDO:0005045)

Orphanet (12): Familial isolated dilated cardiomyopathy (Orphanet:154), BAG3-related myofibrillar myopathy (Orphanet:199340), Rare cardiomyopathy (Orphanet:167848), Familial dilated cardiomyopathy (Orphanet:217607), Dilated cardiomyopathy (Orphanet:217604), Rare familial disorder with hypertrophic cardiomyopathy (Orphanet:99739), Left ventricular noncompaction (Orphanet:54260), Inherited isolated arrhythmogenic cardiomyopathy (Orphanet:217656), Myofibrillar myopathy (Orphanet:593), Rare hypertrophic cardiomyopathy (Orphanet:217569), NON RARE IN EUROPE: Familial isolated hypertrophic cardiomyopathy (Orphanet:155), NON RARE IN EUROPE: Unexplained intellectual disability (Orphanet:319658)

HPO phenotypes

96 total (30 of 96 shown, HPO-id order):

HPO	Term
HP:0000006	Autosomal dominant inheritance
HP:0000365	Hearing impairment
HP:0000407	Sensorineural hearing impairment
HP:0000762	Decreased nerve conduction velocity
HP:0000969	Edema
HP:0001265	Hyporeflexia
HP:0001288	Gait disturbance
HP:0001611	Hypernasal speech
HP:0001635	Congestive heart failure
HP:0001639	Hypertrophic cardiomyopathy
HP:0001644	Dilated cardiomyopathy
HP:0001653	Mitral regurgitation
HP:0001712	Left ventricular hypertrophy
HP:0001723	Restrictive cardiomyopathy
HP:0001727	Thromboembolic stroke
HP:0001761	Pes cavus
HP:0001771	Achilles tendon contracture
HP:0001962	Palpitations
HP:0002063	Rigidity
HP:0002091	Restrictive ventilatory defect
HP:0002093	Respiratory insufficiency
HP:0002166	Impaired vibration sensation in the lower limbs
HP:0002380	Fasciculations
HP:0002460	Distal muscle weakness
HP:0002505	Loss of ambulation
HP:0002522	Areflexia of lower limbs
HP:0002650	Scoliosis
HP:0002875	Exertional dyspnea
HP:0002936	Distal sensory impairment
HP:0002943	Thoracic scoliosis

GWAS associations

34 associations (top):

Study	Trait	p-value
GCST001023_1	Idiopathic dilated cardiomyopathy	4.000000e-12
GCST003984_9	Parkinson’s disease	3.000000e-11
GCST004902_2	Parkinson’s disease	2.000000e-19
GCST007094_64	Diastolic blood pressure	3.000000e-09
GCST009325_3	Parkinson’s disease or first degree relation to individual with Parkinson’s disease	2.000000e-11
GCST009541_10	Heart failure	4.000000e-09
GCST010125_1	Left ventricular ejection fraction	4.000000e-32
GCST010130_2	Left ventricular end-systolic volume	8.000000e-32
GCST010617_1	Left ventricular ejection fraction	8.000000e-17
GCST010796_2009	Electrocardiogram morphology (amplitude at temporal datapoints)	6.000000e-49
GCST010796_2010	Electrocardiogram morphology (amplitude at temporal datapoints)	3.000000e-52
GCST010796_2011	Electrocardiogram morphology (amplitude at temporal datapoints)	2.000000e-46
GCST010796_2012	Electrocardiogram morphology (amplitude at temporal datapoints)	3.000000e-38
GCST010796_2013	Electrocardiogram morphology (amplitude at temporal datapoints)	5.000000e-28
GCST010796_2014	Electrocardiogram morphology (amplitude at temporal datapoints)	8.000000e-20
GCST010796_2015	Electrocardiogram morphology (amplitude at temporal datapoints)	7.000000e-14
GCST010796_2016	Electrocardiogram morphology (amplitude at temporal datapoints)	7.000000e-10
GCST010796_2017	Electrocardiogram morphology (amplitude at temporal datapoints)	6.000000e-09
GCST010796_2018	Electrocardiogram morphology (amplitude at temporal datapoints)	5.000000e-54
GCST010991_10	Parkinson’s disease	2.000000e-12
GCST011198_7	Left ventricular end-systolic volume	3.000000e-18
GCST011202_3	Dilated cardiomyopathy (MTAG)	7.000000e-38
GCST011205_7	Hypertrophic cardiomyopathy (MTAG)	1.000000e-24
GCST011206_3	Left ventricular end-diastolic volume	2.000000e-06
GCST011209_5	Left ventricular ejection fraction	4.000000e-18
GCST011210_3	Dilated cardiomyopathy	2.000000e-32
GCST011211_3	Hypertrophic cardiomyopathy	2.000000e-38
GCST011216_6	Left ventricular global radial strain	2.000000e-08
GCST011217_8	Left ventricular global circumferential strain	6.000000e-20
GCST012099_8	Hypertrophic cardiomyopathy (sarcomere negative)	2.000000e-27

EFO canonical traits (6, from GWAS)

EFO ID	Trait name
EFO:0006336	diastolic blood pressure
EFO:0008373	left ventricular ejection fraction measurement
EFO:0008206	left ventricular systolic function measurement
EFO:0004327	electrocardiography
EFO:0008204	left ventricular diastolic function measurement
EFO:0004736	aspartate aminotransferase measurement

MeSH disease descriptors (11)

Descriptor	Name	Tree numbers
D001281	Atrial Fibrillation	C14.280.067.198; C23.550.073.198
D009202	Cardiomyopathies	C14.280.238
D002311	Cardiomyopathy, Dilated	C14.280.195.160; C14.280.238.070; C16.320.488.750
D002312	Cardiomyopathy, Hypertrophic	C14.280.238.100; C14.280.484.048.750.070.160
D024741	Cardiomyopathy, Hypertrophic, Familial	C14.280.238.100.500; C14.280.484.048.750.070.160.500; C16.320.160
D008607	Intellectual Disability	C10.597.606.360; C23.888.592.604.646; F01.700.687; F03.625.539
D008133	Long QT Syndrome	C14.280.067.565; C14.280.123.625; C16.131.240.400.715; C23.550.073.547
D009205	Myocarditis	C14.280.238.625
C580316	Myofibrillar Myopathy (supp.)
C567843	Myopathy, Myofibrillar, Bag3-Related (supp.)
C536231	familial dilated cardiomyopathy (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (1): CHEMBL5465257 (SINGLE PROTEIN)

PharmGKB: 1 entry (VIP=true, CPIC=false)

ChEMBL bioactivities

4 potent at pChembl≥5 of 13 total, top 4 by pChembl (potency: 10 = 0.1 nM, 6 = 1 µM).

pChembl	Type	Value	Unit	Molecule
6.75	Kd	180	nM	CHEMBL5410389
5.59	Kd	2580	nM	CHEMBL6145087
5.44	Kd	3620	nM	CHEMBL6167778
5.40	Kd	4010	nM	CHEMBL6145087

CTD chemical–gene interactions

130 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects cotreatment, increases expression, affects expression	8
sodium arsenite	affects cotreatment, increases expression, increases abundance	5
methylmercuric chloride	increases expression, affects cotreatment	4
Cadmium	increases abundance, increases expression	4
Estradiol	decreases expression, increases expression	4
Tobacco Smoke Pollution	affects expression, increases expression	4
Bortezomib	increases expression	3
Arsenic	affects expression, increases expression, affects cotreatment, increases abundance, increases reaction	3
Cadmium Chloride	increases abundance, increases expression, affects localization	3
bisphenol A	decreases expression, increases expression	2
zinc chloride	affects localization, increases expression	2
cobaltous chloride	increases expression	2
mercuric bromide	increases expression, affects cotreatment	2
Benzo(a)pyrene	increases expression	2
Dactinomycin	affects cotreatment, increases secretion, decreases reaction, increases expression	2
Doxorubicin	affects expression, increases expression	2
Drugs, Chinese Herbal	decreases expression, increases expression	2
Ivermectin	affects cotreatment, increases expression, decreases expression	2
Phenylmercuric Acetate	affects cotreatment, increases expression	2
Silver	increases expression	2
Zinc	affects cotreatment, increases expression	2
Cyclosporine	increases expression	2
p-Chloromercuribenzoic Acid	increases expression, affects cotreatment	2
Particulate Matter	increases abundance, affects expression, increases reaction, decreases expression	2
aristolochic acid I	increases expression	1
FR900359	affects phosphorylation	1
bisphenol F	increases expression	1
dicrotophos	increases expression	1
2,4,6-tribromophenol	decreases expression	1
bis(tri-n-butyltin)oxide	increases expression	1

ChEMBL screening assays

8 unique, capped per target: 8 binding

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL5336669	Binding	Binding affinity to CM4 sensorchip immobilized C-terminal His tagged human BAG3 transfected in HEK293T cells assessed as binding capacity at 50 uM by SPR analysis	Design, synthesis and biological evaluation of novel 2,4-thiazolidinedione derivatives able to target the human BAG3 protein. — Eur J Med Chem

Cellosaurus cell lines

16 cell lines: 12 induced pluripotent stem cell, 3 cancer cell line, 1 transformed cell line

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_C6SK	SCVIi073-A	Induced pluripotent stem cell	Female
CVCL_C6SL	SCVIi074-A	Induced pluripotent stem cell	Female
CVCL_D6M9	ICANi002-A-3	Induced pluripotent stem cell	Male
CVCL_D6MA	ICANi002-A-4	Induced pluripotent stem cell	Male
CVCL_D8ZZ	Ubigene HEK293 BAG3 KO	Transformed cell line	Female
CVCL_D9YF	Ubigene HeLa BAG3 KO	Cancer cell line	Female
CVCL_E8WH	UKBi022-A	Induced pluripotent stem cell	Male
CVCL_E8WI	UKBi022-A-1	Induced pluripotent stem cell	Male
CVCL_E8WJ	UKBi023-A	Induced pluripotent stem cell	Male
CVCL_E8WK	UKBi023-A-1	Induced pluripotent stem cell	Male

Clinical trials (associated diseases)

301 trials via MONDO — disease-level, not drug-specific.

Trial	Phase	Status	Title
NCT00348530	PHASE4	UNKNOWN	Carvedilol Versus Verapamil in Chronic Heart Failure Secondary to Non-Ischemic Cardiomyopathy
NCT00371891	PHASE4	COMPLETED	Ontario Multidetector Computed Tomographic (MDCT) Coronary Angiography Study (OMCAS)
NCT00401856	PHASE4	COMPLETED	CMR to Assess Fibrosis in Cardiomyopathy Using Eplerenone
NCT00559338	PHASE4	COMPLETED	Impact of Nesiritide Infusion for Decompensated Heart Failure in the Emergency Department
NCT00606775	PHASE4	UNKNOWN	The Preventive Efficacy of Carvedilol on Cardiac Dysfunction in Duchenne Muscular Dystrophy
NCT00658203	PHASE4	COMPLETED	Clinical Evaluation on Advanced Resynchronization
NCT00701220	PHASE4	COMPLETED	Statin Therapy for Ischemic and Nonischemic Cardiomyopathy
NCT00800761	PHASE4	COMPLETED	Intensive Combined Chelation Therapy for Iron-Induced Cardiac Disease in Patients With Thalassemia Major
NCT00806390	PHASE4	TERMINATED	Prevention of Anthracycline or Trastuzumab Induced Cardiomyopathy by Metoprolol
NCT01006473	PHASE4	COMPLETED	Exercise Training in Chagas Cardiomyopathy
NCT01261065	PHASE4	COMPLETED	Mechanisms of Improvement With Beta-Blocker Treatment in Heart Failure
NCT01345188	PHASE4	COMPLETED	Ranolazine in Ischemic Cardiomyopathy
NCT01868841	PHASE4	COMPLETED	123-I mIBG (AdreView) Heart-to-Mediastinal (H/M) Ratio and SPECT Imaging on a Small Field of View-High Efficiency Cardiac SPECT System
NCT02640846	PHASE4	UNKNOWN	Effects of Levosimendan, Milrinone and Norepinephrine on Left and Right Ventricular Function in Septic Shock
NCT03228823	PHASE4	UNKNOWN	Prospective Assessment of Premature Ventricular Contractions Suppression in Cardiomyopathy(PAPS)
NCT04323852	PHASE4	COMPLETED	Can Vitamin D Reduce Heart Muscle Damage After Bypass Surgery?
NCT05034432	PHASE4	RECRUITING	The PIVATAL Study -Study of Ventricular Arrhythmia (VTA) Ablation in Left Ventricular Assist Device (LVAD) Patients
NCT05718128	PHASE4	RECRUITING	Clinical Study of Endocardial Myocardial Biopsy
NCT06964464	PHASE4	RECRUITING	Comparative Effectiveness of Carvedilol Versus Metoprolol Succinate in Heart Failure Patients With an Implantable Cardioverter Defibrillator
NCT00170183	PHASE3	COMPLETED	Brain Natriuretic Peptide (BNP) to Preserve Renal Function in Hospitalized Patients With Heart Failure
NCT00270387	PHASE3	COMPLETED	A Study of Short-Term Outcomes and Economic Impact For Patients With Worsening Congestive Heart Failure When Natrecor (Nesiritide) is Added to Standard-Care Therapy, Compared to Administration of Placebo With Standard-Care Therapy
NCT00321295	PHASE3	COMPLETED	Biventricular Pacing In Patients With Left Ventricular Dysfunction After Cardiovascular Surgery
NCT00483197	PHASE3	UNKNOWN	VentrAssistTM LVAD as a Bridge to Cardiac Transplantation - Pivotal Trial
NCT00490321	PHASE3	UNKNOWN	VentrAssistTM LVAD for the Treatment of Advanced Heart Failure - Destination Therapy
NCT00626028	PHASE3	COMPLETED	Comparison of Inhaled Nitric Oxide and Oxygen in Participants Reactivity During Acute Pulmonary Vasodilator Testing
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