TIMM8B

Summary

TIMM8B (translocase of inner mitochondrial membrane 8 homolog B, HGNC:11818) is a protein-coding gene on chromosome 11q23.1, encoding Mitochondrial import inner membrane translocase subunit Tim8 B (Q9Y5J9). Probable mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane.

This gene encodes a member of a well-conserved family of proteins with similarity to yeast Tim mitochondrial import proteins. This gene is encoded by a nuclear gene and is transported into the intermembrane space of the mitochondrion. When formed into complexes, these proteins guide membrane-spanning proteins across the mitochondrial intermembrane space before they are added into the mitochondrial inner membrane. This gene is adjacent to succinate dehydrogenase, subunit D (SDHD), in which mutations have been found in affected members of families with hereditary paraganglioma.

Source: NCBI Gene 26521 — RefSeq curated summary.

At a glance

• Clinical variants (ClinVar): 15 total — 1 pathogenic
• MANE Select transcript: NM_012459

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:11818
Approved symbol	TIMM8B
Name	translocase of inner mitochondrial membrane 8 homolog B
Location	11q23.1
Locus type	gene with protein product
Status	Approved
Aliases	TIM8B, DDP2, FLJ21744, MGC102866, MGC117373
Ensembl gene	ENSG00000150779
Ensembl biotype	protein_coding
OMIM	606659
Entrez	26521

Gene structure

Transcript identifiers

Ensembl transcripts: 4 — 2 protein_coding, 1 protein_coding_CDS_not_defined, 1 nonsense_mediated_decay

ENST00000504148, ENST00000507614, ENST00000509359, ENST00000541231

RefSeq mRNA: 1 — MANE Select: NM_012459 NM_012459

CCDS: CCDS8357

Canonical transcript exons

ENST00000504148 — 2 exons

Exon	Start	End
ENSE00002192689	112086640	112086756
ENSE00003631376	112084800	112085462

Expression profiles

Bgee: expression breadth ubiquitous, 288 present calls, max score 99.33.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 79.3152 / max 405.6633, expressed in 1826 samples.

FANTOM5 promoters (3 alternative TSS)

Promoter ID	TPM avg	Samples expressed
122306	78.5558	1826
122308	0.3846	179
122307	0.3748	181

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
tongue squamous epithelium	UBERON:0006919	99.33	gold quality
heart right ventricle	UBERON:0002080	99.31	gold quality
cervix squamous epithelium	UBERON:0006922	99.21	gold quality
body of tongue	UBERON:0011876	99.00	gold quality
diaphragm	UBERON:0001103	98.96	gold quality
gingival epithelium	UBERON:0001949	98.96	gold quality
pharyngeal mucosa	UBERON:0000355	98.94	gold quality
skeletal muscle tissue of rectus abdominis	UBERON:0004511	98.93	gold quality
skeletal muscle tissue of biceps brachii	UBERON:0004502	98.89	gold quality
biceps brachii	UBERON:0001507	98.88	gold quality
gingiva	UBERON:0001828	98.78	gold quality
triceps brachii	UBERON:0001509	98.74	gold quality
cervix epithelium	UBERON:0004801	98.74	gold quality
tongue	UBERON:0001723	98.73	gold quality
periodontal ligament	UBERON:0008266	98.67	gold quality
oral cavity	UBERON:0000167	98.64	gold quality
mammalian vulva	UBERON:0000997	98.57	gold quality
squamous epithelium	UBERON:0006914	98.53	gold quality
gluteal muscle	UBERON:0002000	98.51	gold quality
superior surface of tongue	UBERON:0007371	98.42	gold quality
vena cava	UBERON:0004087	98.33	gold quality
hair follicle	UBERON:0002073	98.30	gold quality
pigmented layer of retina	UBERON:0001782	98.25	gold quality
penis	UBERON:0000989	98.24	gold quality
epithelium of esophagus	UBERON:0001976	98.16	gold quality
esophagus squamous epithelium	UBERON:0006920	98.15	gold quality
cardiac ventricle	UBERON:0002082	98.12	gold quality
heart left ventricle	UBERON:0002084	98.08	gold quality
nipple	UBERON:0002030	98.07	gold quality
apex of heart	UBERON:0002098	97.92	gold quality

Single-cell (SCXA)

Detected in 2 experiment(s), a significant marker in 1.

Experiment	Marker?	Max mean expression
E-ANND-3	yes	14.20
E-MTAB-8271	no	7.04

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

50 targeting TIMM8B, 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-29A-3P	100.00	73.11	1835
HSA-MIR-29B-3P	100.00	73.18	1833
HSA-MIR-29C-3P	100.00	73.15	1833
HSA-MIR-4776-3P	100.00	68.73	1340
HSA-MIR-507	99.97	70.11	1915
HSA-MIR-557	99.96	70.01	1640
HSA-MIR-9983-3P	99.94	71.48	3631
HSA-MIR-10527-5P	99.91	72.28	3754
HSA-MIR-5682	99.89	72.56	1005
HSA-MIR-129-5P	99.88	70.26	3273
HSA-MIR-1323	99.83	69.89	2471
HSA-MIR-4659A-3P	99.80	72.62	4248
HSA-MIR-4659B-3P	99.80	72.62	4248
HSA-MIR-320A-3P	99.77	69.73	2107
HSA-MIR-320B	99.77	69.73	2107
HSA-MIR-320C	99.77	69.73	2107
HSA-MIR-320D	99.77	69.73	2107
HSA-MIR-4429	99.77	69.62	2111
HSA-MIR-4729	99.69	72.18	4233
HSA-MIR-10393-5P	99.65	68.01	1368
HSA-MIR-4756-3P	99.62	66.30	1319
HSA-MIR-6727-3P	99.49	65.92	1333
HSA-MIR-6871-3P	99.43	68.85	741
HSA-MIR-135A-5P	99.36	71.85	1601
HSA-MIR-135B-5P	99.36	71.63	1613
HSA-MIR-4722-3P	99.35	65.22	1099
HSA-MIR-3940-5P	99.14	65.26	493
HSA-MIR-4507	99.14	65.27	515
HSA-MIR-5583-3P	99.06	65.68	1018
HSA-MIR-4686	98.77	66.87	964

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	timm8b	ENSDARG00000055708
mus_musculus	Timm8b	ENSMUSG00000039016
rattus_norvegicus	Timm8b	ENSRNOG00000009888
drosophila_melanogaster	Tim8	FBGN0027359

Paralogs (1): TIMM8A (ENSG00000126953)

Protein

Protein identifiers

Mitochondrial import inner membrane translocase subunit Tim8 B — Q9Y5J9 (reviewed: Q9Y5J9)

Alternative names: DDP-like protein, Deafness dystonia protein 2

All UniProt accessions (3): Q9Y5J9, E9PIR3, G3XAN8

UniProt curated annotations — full annotation on UniProt →

Function. Probable mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space.

Subunit / interactions. Heterohexamer; possibly composed of 3 copies of TIMM8B and 3 copies of TIMM13, named soluble 70 kDa complex. Associates with the TIM22 complex, whose core is composed of TIMM22.

Subcellular location. Mitochondrion inner membrane.

Tissue specificity. Ubiquitous, with highest expression in heart, kidney, liver and skeletal muscle.

Domain organisation. The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM8B from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane.

Similarity. Belongs to the small Tim family.

RefSeq proteins (1): NP_036591* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR004217	Tim10-like	Domain
IPR035427	Tim10-like_dom_sf	Homologous_superfamily

Pfam: PF02953

UniProt features (6 total): disulfide bond 2, initiator methionine 1, chain 1, short sequence motif 1, modified residue 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q9Y5J9-F1	93.60	0.87

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 (1): 2

Disulfide bonds (2): 36–59, 40–55

Function

Pathways and Gene Ontology

Reactome pathways

1 pathways

ID	Pathway
R-HSA-1268020	Mitochondrial protein import

MSigDB gene sets: 187 (showing top): BUYTAERT_PHOTODYNAMIC_THERAPY_STRESS_DN, GOBP_SENSORY_PERCEPTION_OF_MECHANICAL_STIMULUS, STARK_PREFRONTAL_CORTEX_22Q11_DELETION_DN, GOBP_PROTEIN_TARGETING, TGACCTY_ERR1_Q2, GOBP_ESTABLISHMENT_OF_PROTEIN_LOCALIZATION_TO_ORGANELLE, GOBP_INNER_MITOCHONDRIAL_MEMBRANE_ORGANIZATION, GOBP_ESTABLISHMENT_OF_PROTEIN_LOCALIZATION_TO_MEMBRANE, GOCC_MITOCHONDRIAL_ENVELOPE, YAO_TEMPORAL_RESPONSE_TO_PROGESTERONE_CLUSTER_13, LASTOWSKA_NEUROBLASTOMA_COPY_NUMBER_DN, TIEN_INTESTINE_PROBIOTICS_24HR_UP, GOBP_PROTEIN_LOCALIZATION_TO_ORGANELLE, GOBP_SENSORY_PERCEPTION, NRF2_01

GO Biological Process (4): obsolete protein targeting to mitochondrion (GO:0006626), sensory perception of sound (GO:0007605), protein transport (GO:0015031), protein insertion into mitochondrial inner membrane (GO:0045039)

GO Molecular Function (3): zinc ion binding (GO:0008270), protein transporter activity (GO:0140318), metal ion binding (GO:0046872)

GO Cellular Component (6): obsolete extracellular space (GO:0005615), mitochondrion (GO:0005739), mitochondrial inner membrane (GO:0005743), mitochondrial intermembrane space (GO:0005758), mitochondrial intermembrane space chaperone complex (GO:0042719), membrane (GO:0016020)

Reactome top-level categories

Rollup of top-1 pathways:

Category	Pathways
Protein localization	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
sensory perception of mechanical stimulus	1
transport	1
intracellular protein localization	1
establishment of protein localization	1
inner mitochondrial membrane organization	1
mitochondrial protein import pathway	1
transition metal ion binding	1
transporter activity	1
cation binding	1
cytoplasm	1
intracellular membrane-bounded organelle	1
organelle inner membrane	1
mitochondrial membrane	1
mitochondrial envelope	1
organelle envelope lumen	1
mitochondrial intermembrane space	1
mitochondrial protein-containing complex	1
cellular anatomical structure	1

Protein interactions and networks

STRING

1448 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
TIMM8B	SDHD	O14521	871
TIMM8B	DPP7	Q9UHL4	844
TIMM8B	TIMM13	P62206	783
TIMM8B	SDHC	Q99643	734
TIMM8B	SDHB	P21912	703
TIMM8B	TIMM10B	Q9Y5J6	690
TIMM8B	TIMM9	Q9Y5J7	665
TIMM8B	TIMM10	P62072	644
TIMM8B	TIMM17A	Q99595	628
TIMM8B	TOMM22	Q9NS69	579
TIMM8B	TIMM22	Q9Y584	572
TIMM8B	TOMM7	Q9P0U1	572
TIMM8B	NKAPD1	Q6ZUT1	570
TIMM8B	TIMM17B	O60830	549
TIMM8B	MSTO1	Q9BUK6	521

IntAct

59 interactions, top by confidence:

A	B	Type	Score
AFG2B	AFG2A	psi-mi:“MI:0914”(association)	0.800
CCDC120	AIP	psi-mi:“MI:0914”(association)	0.640
KLK5	DENND11	psi-mi:“MI:0914”(association)	0.640
KLC2	KIF5B	psi-mi:“MI:0914”(association)	0.530
PDYN	ACAD11	psi-mi:“MI:0914”(association)	0.530
ORF10	PPT1	psi-mi:“MI:0914”(association)	0.530
YBEY	NME4	psi-mi:“MI:0914”(association)	0.530
env	PGRMC1	psi-mi:“MI:0914”(association)	0.460
AIFM1	HAX1	psi-mi:“MI:0914”(association)	0.420
EGFL8	TIMM8B	psi-mi:“MI:0915”(physical association)	0.370
TIMM8B	HADHB	psi-mi:“MI:0915”(physical association)	0.370
MYO1C	PLEKHG3	psi-mi:“MI:0914”(association)	0.350
GPATCH8	FDX1	psi-mi:“MI:0914”(association)	0.350
FOXI2	DDX39A	psi-mi:“MI:0914”(association)	0.350
MAP3K14	IKBKG	psi-mi:“MI:0914”(association)	0.350
P2RY6	ESYT2	psi-mi:“MI:0914”(association)	0.350
SLC15A3		psi-mi:“MI:0914”(association)	0.350
TCF7L2	LOC401309	psi-mi:“MI:0914”(association)	0.350
P2RY6		psi-mi:“MI:0914”(association)	0.350
YBEY	NUDT19	psi-mi:“MI:0914”(association)	0.350
NMES1	COX7A2L	psi-mi:“MI:0914”(association)	0.350
COQ10B	ALDH1L1	psi-mi:“MI:0914”(association)	0.350
	CLGN	psi-mi:“MI:0914”(association)	0.350
	TIMM8A	psi-mi:“MI:0914”(association)	0.350
BCKDK	AIP	psi-mi:“MI:0914”(association)	0.350
STRADA		psi-mi:“MI:0914”(association)	0.350

BioGRID (72): TIMM8B (Affinity Capture-MS), TIMM8B (Co-fractionation), TIMM8B (Co-fractionation), TIMM8B (Co-fractionation), TIMM8B (Co-fractionation), TIMM8B (Co-fractionation), TIMM8B (Co-fractionation), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS), TIMM8B (Affinity Capture-MS)

ESM2 similar proteins: A4QNC6, B0BN94, O74700, P0CR96, P0CR97, P0CS00, P0CS01, P30629, P62075, P62076, P62077, P62078, Q10481, Q2HJI3, Q2KIV2, Q3SZ93, Q4I6B0, Q4V7R1, Q59MI8, Q59R24, Q5ZIR8, Q616Q2, Q61TH2, Q63ZH8, Q66L32, Q6BN23, Q6BU42, Q6CM57, Q6DEM5, Q6DGJ3, Q6FK81, Q6GPY0, Q6PBU0, Q75DU7, Q7SBR3, Q8AVK1, Q90YI5, Q96C01, Q9CR98, Q9N408

Diamond homologs: O60220, P0CR94, P0CR95, P57744, P62075, P62076, P62077, P62078, Q3SZ93, Q3ZBS8, Q4FZG7, Q4I6B0, Q59MI8, Q616Q2, Q66L32, Q6BN23, Q6CWH5, Q6DEM5, Q6DGJ3, Q6FK81, Q6GPY0, Q75DU7, Q8AVK1, Q90YI5, Q9N408, Q9VTN3, Q9WVA1, Q9WVA2, Q9XGY4, Q9Y1A3, Q9Y5J9, Q9Y5L4, Q9Y8C0, O45319, Q09783, Q4IJW4, Q3SZW4, Q5RDJ0, Q6BHJ3, Q6CIK7

SIGNOR signaling

0 interactions.

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 78 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
Complex IV assembly	5	22.4×	8e-04

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	1
Likely pathogenic	0
Uncertain significance	13
Likely benign	0
Benign	0

Top pathogenic / likely-pathogenic (1)

Variant ID	HGVS	Classification
1076623	NC_000011.9:g.(?111956548)(111967525_?)del	Pathogenic

SpliceAI

273 predictions. Top by Δscore:

Variant	Effect	Δscore
11:112086169:T:TA	donor_gain	1.0000
11:112086635:CGCA:C	donor_loss	1.0000
11:112086636:GCAC:G	donor_loss	1.0000
11:112086637:CACC:C	donor_loss	1.0000
11:112086639:C:CT	donor_loss	1.0000
11:112085461:ACCTA:A	acceptor_loss	0.9900
11:112085462:CCTAA:C	acceptor_loss	0.9900
11:112085463:CTAAA:C	acceptor_loss	0.9900
11:112085464:T:A	acceptor_loss	0.9900
11:112085976:A:AC	donor_gain	0.9900
11:112085977:C:CC	donor_gain	0.9900
11:112086045:C:A	donor_gain	0.9900
11:112086147:T:TA	donor_gain	0.9900
11:112086182:T:A	donor_gain	0.9900
11:112085463:C:CC	acceptor_gain	0.9800
11:112086044:T:TA	donor_gain	0.9800
11:112086164:C:CT	donor_gain	0.9800
11:112086165:T:TT	donor_gain	0.9800
11:112086656:G:A	donor_gain	0.9800
11:112085458:TGCAC:T	acceptor_gain	0.9700
11:112086660:T:TA	donor_gain	0.9600
11:112086017:AGGT:A	donor_gain	0.9500
11:112086225:ATGAT:A	donor_gain	0.9500
11:112086653:TG:T	donor_gain	0.9500
11:112086176:C:CT	donor_gain	0.9400
11:112086177:C:CT	donor_gain	0.9400
11:112085460:CAC:C	acceptor_gain	0.9300
11:112085977:CTAT:C	donor_gain	0.9300
11:112086129:CCAA:C	donor_gain	0.9300
11:112086166:A:AC	donor_gain	0.9300

AlphaMissense

547 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
11:112085436:C:A	W37C	0.998
11:112085436:C:G	W37C	0.998
11:112085358:G:C	F63L	0.996
11:112085358:G:T	F63L	0.996
11:112085360:A:G	F63L	0.996
11:112085362:C:G	R62P	0.995
11:112085440:C:T	C36Y	0.994
11:112086657:C:G	A23P	0.994
11:112085356:A:T	I64N	0.993
11:112085359:A:G	F63S	0.993
11:112085371:C:T	C59Y	0.993
11:112085380:A:G	L56P	0.993
11:112085342:C:G	A69P	0.992
11:112085439:A:C	C36W	0.992
11:112085338:A:T	I70N	0.991
11:112085383:C:T	C55Y	0.991
11:112086645:C:G	A27P	0.991
11:112086650:A:G	F25S	0.991
11:112085372:A:G	C59R	0.990
11:112085380:A:T	L56H	0.990
11:112085428:C:G	C40S	0.990
11:112085429:A:T	C40S	0.990
11:112085326:A:G	F74S	0.989
11:112085329:C:G	R73P	0.989
11:112085353:T:A	D65V	0.989
11:112085429:A:G	C40R	0.989
11:112085440:C:A	C36F	0.989
11:112085440:C:G	C36S	0.989
11:112085441:A:G	C36R	0.989
11:112085441:A:T	C36S	0.989

dbSNP variants (sampled 300 via entrez): RS1000545537 (11:112088580 C>G), RS1001883229 (11:112085144 CTT>C), RS1002020406 (11:112084889 A>C), RS1002274236 (11:112088515 C>A,T), RS1002347725 (11:112088348 C>G,T), RS1003320468 (11:112087121 A>C), RS1003347831 (11:112086776 G>A,T), RS1004627553 (11:112086167 C>T), RS1005260403 (11:112084797 GC>G), RS1005272147 (11:112084489 G>A,T), RS1007402439 (11:112084513 G>A), RS1008619752 (11:112087219 C>A,G), RS1009361834 (11:112087588 G>A), RS1010167356 (11:112088723 A>C,G), RS1011825806 (11:112085924 G>A,C,T)

Disease associations

OMIM: gene MIM:606659 | disease phenotypes: MIM:171300, MIM:606864, MIM:615106

GenCC curated gene-disease

Mondo (3): pheochromocytoma (MONDO:0008233), Carney-Stratakis syndrome (MONDO:0011740), Cowden syndrome 3 (MONDO:0014045)

Orphanet (3): Cowden syndrome (Orphanet:201), Hereditary pheochromocytoma-paraganglioma (Orphanet:29072), Carney-Stratakis syndrome (Orphanet:97286)

HPO phenotypes

0 total (0 of 0 shown, HPO-id order):

GWAS associations

0 associations (top):

MeSH disease descriptors (2)

Descriptor	Name	Tree numbers
D010673	Pheochromocytoma	C04.557.465.625.650.700.725; C04.557.580.625.650.700.725
C564650	Carney-Stratakis Syndrome (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

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

CTD chemical–gene interactions

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

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects cotreatment, increases expression, affects expression, decreases expression	5
bisphenol A	decreases expression, increases expression, affects expression	3
sodium arsenite	decreases expression, increases expression	2
GSK-J4	decreases expression	1
FR900359	increases phosphorylation	1
triphenyl phosphate	affects expression	1
nobiletin	decreases expression, decreases reaction	1
sodium arsenate	decreases expression, decreases reaction	1
O,O-diethyl O-3,5,6-trichloro-2-pyridyl phosphate	affects expression, affects response to substance	1
beta-lapachone	increases expression	1
methylparaben	decreases expression	1
tris(1,3-dichloro-2-propyl)phosphate	decreases expression	1
cobaltous chloride	decreases expression	1
perfluorooctanoic acid	increases expression	1
ochratoxin A	decreases expression	1
di-n-butylphosphoric acid	affects expression	1
CGP 52608	affects binding, increases reaction	1
CD 437	decreases expression	1
K 7174	decreases expression	1
4-(5-benzo(1,3)dioxol-5-yl-4-pyridin-2-yl-1H-imidazol-2-yl)benzamide	affects cotreatment, decreases expression	1
3-(4’-hydroxy-3’-adamantylbiphenyl-4-yl)acrylic acid	decreases expression	1
abrine	decreases expression	1
LDN 193189	affects cotreatment, decreases expression	1
Temozolomide	increases expression	1
Sunitinib	decreases expression	1
Air Pollutants	decreases expression, increases abundance	1
Arsenic	affects methylation	1
Cannabidiol	increases expression	1
Dexamethasone	increases expression	1
Diazinon	increases methylation	1

Clinical trials (associated diseases)

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

Trial	Phase	Status	Title
NCT01379898	PHASE4	COMPLETED	Phenoxybenzamine Versus Doxazosin in PCC Patients
NCT01959711	PHASE4	COMPLETED	Randomized Clinical Trial of Posterior Retroperitoneoscopic Adrenalectomy Versus Lateral Laparoscopic Adrenalectomy
NCT05702944	PHASE4	RECRUITING	The Effect and Safety of Omitting Preoperative Alpha-adrenergic Blockade for Normotensive Pheochromocytoma
NCT00002641	PHASE3	COMPLETED	Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma
NCT00002764	PHASE3	COMPLETED	Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma
NCT00126412	PHASE3	COMPLETED	Meta-Iodobenzylguanidine (123I mIBG) Scintigraphy in Patients Being Evaluated for Phaeochromocytoma or Neuroblastoma
NCT01373736	PHASE3	UNKNOWN	123I-MIBG Scintigraphy in Patients Being Evaluated for Neuroendocrine Tumors
NCT03176693	PHASE3	COMPLETED	Preoperative Alpha Blockade for Pheochromocytoma
NCT00002608	PHASE2	COMPLETED	Combination Chemotherapy and Tamoxifen in Treating Patients With Solid Tumors
NCT00028106	PHASE2	COMPLETED	131MIBG to Treat Malignant Pheochromocytoma
NCT00107289	PHASE2	RECRUITING	Iodine I 131 Metaiodobenzylguanidine in Treating Patients With Recurrent, Progressive, or Refractory Neuroblastoma or Malignant Pheochromocytoma or Paraganglioma
NCT00466856	PHASE2	TERMINATED	Internal Radiation Therapy in Treating Patients With Liver Metastases From Neuroendocrine Tumors
NCT00843037	PHASE2	COMPLETED	Study Of Sunitinib In Patients With Recurrent Paraganglioma/Pheochromocytoma
NCT00874614	PHASE2	UNKNOWN	A Study Evaluating Ultratrace Iobenguane I131 in Patients With Malignant Relapsed/Refractory Pheochromocytoma/Paraganglioma
NCT00923481	PHASE2	COMPLETED	A Broad Multi-histology Phase II Study of the Multi-Kinase Inhibitor R935788 (Fostamatinib Disodium) in Advanced Colorectal, Non-small Cell Lung, Head and Neck Hepatocellular and Renal Cell Carcinomas, and Pheochromocytoma and Thyroid Tumors (Multi-H…
NCT01152827	PHASE2	COMPLETED	RAD001 in Pheochromocytoma or Nonfunctioning Carcinoid
NCT01413503	PHASE2	COMPLETED	A Phase II Study of 131I- Metaiodobenzylguanidine (MIBG) for Treatment of Metastatic or Unresectable Pheochromocytoma and Related Tumors
NCT01635907	PHASE2	COMPLETED	Dovitinib in Neuroendocrine Tumors
NCT01967576	PHASE2	COMPLETED	Phase II Study of Axitinib (AG-013736) With Evaluation of the VEGF-pathway in Metastatic, Recurrent or Primary Unresectable Pheochromocytoma/Paraganglioma
NCT03165721	PHASE2	TERMINATED	A Phase II Trial of the DNA Methyl Transferase Inhibitor, Guadecitabine (SGI-110), in Children and Adults With Wild Type GIST,Pheochromocytoma and Paraganglioma Associated With Succinate Dehydrogenase Deficiency and HLRCC-associated Kidney Cancer
NCT03206060	PHASE2	RECRUITING	Lu-177-DOTATATE (Lutathera) in Therapy of Inoperable Pheochromocytoma/ Paraganglioma
NCT03839498	PHASE2	ACTIVE_NOT_RECRUITING	Study of Axitinib (AG-013736) With Evaluation of the VEGF-pathway in Pheochromocytoma/Paraganglioma
NCT03946527	PHASE2	ACTIVE_NOT_RECRUITING	LAnreotide in Metastatic Pheochromocytoma / PARAganglioma (LAMPARA)
NCT04276597	PHASE2	WITHDRAWN	Phase-II Study of Lu177DOTATOC in Adults With STTR(+)Pulmonary, Pheochromocytoma, Paraganglioma, Unknown Primary, Thymus NETs (PUTNET), or Any Other Non-.GEP-NET.
NCT04320589	PHASE2	COMPLETED	the Effect of Dexmedetomidine and Magnesium Sulfate in Open Resection of Pheochromocytoma
NCT04400474	PHASE2	COMPLETED	Trial of Cabozantinib Plus Atezolizumab in Advanced and Progressive Neoplasms of the Endocrine System. The CABATEN Study
NCT04711135	PHASE2	ACTIVE_NOT_RECRUITING	Study to Evaluate Safety and Dosimetry of Lutathera in Adolescent Patients With GEP-NETs and PPGLs
NCT04924075	PHASE2	RECRUITING	Belzutifan/MK-6482 for the Treatment of Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Solid Tumors With HIF-2α Related Genetic Alterations (MK-6482-015)
NCT05133349	PHASE2	UNKNOWN	A Prospective Phase II Efficacy and Safety Study of Anlotinib in Metastatic or Locally Advanced Pheochromocytoma/ Paraganglioma : Open-label Single-arm, Exploratory Trial
NCT05883085	PHASE2	UNKNOWN	A Study on the Safety and Effectiveness of Anlotinib for Neoadjuvant Treatment of PPGL
NCT05885386	PHASE2	UNKNOWN	A Study on the Safety and Effectiveness of Temozolomide for Neoadjuvant Treatment of PPGL
NCT06045260	PHASE2	RECRUITING	Receptor Radionuclide Therapy With 177Lu-DOTATOC
NCT06233903	PHASE2	WITHDRAWN	18F-mFBG Expression in Neural Crest Tumors and Organs Innervated by the Sympathetic Nervous System
NCT06429397	PHASE2	NOT_YET_RECRUITING	Anlotinib Combined With Benmelstobart for Advanced Pheochromocytoma
NCT06503146	PHASE2	RECRUITING	18F-Fibroblast Activation Protein Inhibitor ([18F]FAPI-74) PET Imaging for Cancer Detection
NCT06683846	PHASE2	RECRUITING	Ivonescimab in the Treatment of Multiple Advanced Tumors
NCT07167329	PHASE2	RECRUITING	Real-World Effectiveness and Pharmacogenetics of Belzutifan in VHL Syndrome: The BELIEVE-VHL Trial
NCT00002947	PHASE1	TERMINATED	Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer
NCT00004847	PHASE1	RECRUITING	Diagnosis of Pheochromocytoma
NCT00049023	PHASE1	COMPLETED	Radiolabeled Octreotide in Treating Children With Advanced or Refractory Solid Tumors