EEF2KMT

Summary

EEF2KMT (eukaryotic elongation factor 2 lysine methyltransferase, HGNC:32221) is a protein-coding gene on chromosome 16p13.3, encoding Protein-lysine N-methyltransferase EEF2KMT (Q96G04). Catalyzes the trimethylation of eukaryotic elongation factor 2 (EEF2) on ‘Lys-525’. It is a selective cancer dependency (DepMap: 84.9% of cell lines).

Enables protein-lysine N-methyltransferase activity. Involved in peptidyl-lysine trimethylation. Located in cytoplasm. Part of protein-containing complex.

Source: NCBI Gene 196483 — RefSeq curated summary.

At a glance

• GWAS associations: 5
• Clinical variants (ClinVar): 117 total — 1 pathogenic
• Cancer dependency (DepMap): dependent in 84.9% of screened cell lines
• MANE Select transcript: NM_201400

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:32221
Approved symbol	EEF2KMT
Name	eukaryotic elongation factor 2 lysine methyltransferase
Location	16p13.3
Locus type	gene with protein product
Status	Approved
Aliases	SB153, MGC19636, EFM3
Ensembl gene	ENSG00000118894
Ensembl biotype	protein_coding
OMIM	615263
Entrez	196483

Gene structure

Transcript identifiers

Ensembl transcripts: 14 — 8 protein_coding, 3 nonsense_mediated_decay, 3 retained_intron

ENST00000427587, ENST00000458008, ENST00000585436, ENST00000585975, ENST00000586444, ENST00000587133, ENST00000587161, ENST00000587200, ENST00000587608, ENST00000902788, ENST00000940846, ENST00000940847, ENST00000940848, ENST00000956562

RefSeq mRNA: 3 — MANE Select: NM_201400 NM_001289029, NM_201400, NM_201598

CCDS: CCDS10529, CCDS10530, CCDS73823

Canonical transcript exons

ENST00000427587 — 8 exons

Exon	Start	End
ENSE00002832093	5097644	5097795
ENSE00003483343	5090084	5090349
ENSE00003511143	5095452	5095514
ENSE00003513644	5090432	5090565
ENSE00003540900	5089107	5089256
ENSE00003572713	5091794	5091895
ENSE00003680657	5093484	5093564
ENSE00003894264	5084284	5085732

Expression profiles

Bgee: expression breadth ubiquitous, 182 present calls, max score 85.83.

FANTOM5 (CAGE): breadth broad, TPM avg 0.4829 / max 20.8486, expressed in 248 samples.

FANTOM5 promoters (1 alternative TSS)

Promoter ID	TPM avg	Samples expressed
156146	0.4829	248

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
male germ line stem cell (sensu Vertebrata) in testis	CL:0000089 ∩ UBERON:0000473	85.83	gold quality
primordial germ cell in gonad	CL:0000670 ∩ UBERON:0000991	85.44	gold quality
mucosa of transverse colon	UBERON:0004991	84.30	gold quality
stromal cell of endometrium	CL:0002255	83.77	gold quality
left testis	UBERON:0004533	82.42	gold quality
right testis	UBERON:0004534	82.27	gold quality
right adrenal gland	UBERON:0001233	81.76	gold quality
right adrenal gland cortex	UBERON:0035827	81.43	gold quality
apex of heart	UBERON:0002098	81.24	gold quality
left adrenal gland	UBERON:0001234	81.04	gold quality
left adrenal gland cortex	UBERON:0035825	80.82	gold quality
testis	UBERON:0000473	80.76	gold quality
right lobe of liver	UBERON:0001114	80.50	gold quality
granulocyte	CL:0000094	80.36	gold quality
gastrocnemius	UBERON:0001388	80.07	gold quality
body of pancreas	UBERON:0001150	80.03	gold quality
adenohypophysis	UBERON:0002196	79.62	gold quality
adrenal cortex	UBERON:0001235	79.54	gold quality
body of stomach	UBERON:0001161	79.20	gold quality
muscle of leg	UBERON:0001383	79.18	gold quality
adrenal gland	UBERON:0002369	79.13	gold quality
left coronary artery	UBERON:0001626	79.08	gold quality
left uterine tube	UBERON:0001303	78.94	gold quality
right lobe of thyroid gland	UBERON:0001119	78.72	gold quality
hindlimb stylopod muscle	UBERON:0004252	78.68	gold quality
rectum	UBERON:0001052	78.62	gold quality
transverse colon	UBERON:0001157	78.53	gold quality
right coronary artery	UBERON:0001625	78.53	gold quality
islet of Langerhans	UBERON:0000006	78.26	gold quality
lower esophagus muscularis layer	UBERON:0035833	78.23	gold quality

Single-cell (SCXA)

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

Experiment	Marker?	Max mean expression
E-ANND-3	yes	3.59
E-MTAB-6379	no	73.23

Regulation

Is transcription factor: no

Upstream regulators (CollecTRI, top): NR1I2

miRNA regulators (miRDB)

60 targeting EEF2KMT, 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-4510	100.00	66.60	2050
HSA-MIR-6127	100.00	66.76	2188
HSA-MIR-6129	100.00	66.46	2080
HSA-MIR-6130	100.00	66.69	2012
HSA-MIR-6133	100.00	66.48	2064
HSA-MIR-10401-5P	99.99	65.79	948
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-3143	99.93	71.96	3104
HSA-MIR-4778-3P	99.93	70.40	1818
HSA-MIR-515-5P	99.92	69.82	2343
HSA-MIR-519E-5P	99.92	69.62	2358
HSA-MIR-4648	99.91	67.00	710
HSA-MIR-129-5P	99.88	70.26	3273
HSA-MIR-4492	99.87	68.25	3611
HSA-MIR-5582-3P	99.86	72.48	4221
HSA-MIR-4728-5P	99.85	69.39	4718
HSA-MIR-6785-5P	99.82	68.68	4428
HSA-MIR-6875-3P	99.82	70.26	2983
HSA-MIR-3180-5P	99.82	69.12	2422
HSA-MIR-4659A-3P	99.80	72.62	4248
HSA-MIR-4659B-3P	99.80	72.62	4248
HSA-MIR-149-3P	99.72	68.22	3963
HSA-MIR-6883-5P	99.69	68.05	3785
HSA-MIR-7152-5P	99.60	69.33	2094
HSA-MIR-762	99.58	66.61	1994
HSA-MIR-543	99.52	69.03	2595
HSA-MIR-4498	99.47	67.42	2360
HSA-MIR-12132	99.47	68.90	1341

Functional genomics

DepMap (CRISPR cell-line fitness): dependent in 84.9% of screened cell lines.

Literature-anchored findings (GeneRIF, showing 2)

• Previously uncharacterized lysine-specific methyltransferase FAM86A and Yjr129c introduce a functionally important lysine methylation in eEF2. (PMID:25231979)
• The FAM86 domain of FAM86A confers substrate specificity to promote EEF2-Lys525 methylation. (PMID:37209825)

Cross-species orthologs

5 orthologs

Organism	Symbol	Gene ID
danio_rerio	eef2kmt	ENSDARG00000054950
mus_musculus	Eef2kmt	ENSMUSG00000022544
rattus_norvegicus	Eef2kmt	ENSRNOG00000002876
drosophila_melanogaster	CG7889	FBGN0031003
caenorhabditis_elegans		WBGENE00011144

Paralogs (2): FAM86B2 (ENSG00000145002), FAM86B1 (ENSG00000186523)

Protein

Protein identifiers

Protein-lysine N-methyltransferase EEF2KMT — Q96G04 (reviewed: Q96G04)

Alternative names: eEF2-lysine methyltransferase

All UniProt accessions (4): Q96G04, K7EIJ3, K7EQE4, K7ES84

UniProt curated annotations — full annotation on UniProt →

Function. Catalyzes the trimethylation of eukaryotic elongation factor 2 (EEF2) on ‘Lys-525’.

Subunit / interactions. Interacts with FAM86B2 and FAM86C1P.

Subcellular location. Cytoplasm.

Similarity. Belongs to the class I-like SAM-binding methyltransferase superfamily. EEF2KMT family.

Isoforms (2)

UniProt ID	Names	Canonical?
Q96G04-1	1	yes
Q96G04-2	2

RefSeq proteins (3): NP_001275958, NP_958802, NP_963892 (=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR019410	Methyltransf_16	Family
IPR029063	SAM-dependent_MTases_sf	Homologous_superfamily
IPR029426	FAM86_N	Domain

Pfam: PF10294, PF14904

Catalyzed reactions (Rhea), 1 shown:

• L-lysyl-[protein] + 3 S-adenosyl-L-methionine = N(6),N(6),N(6)-trimethyl-L-lysyl-[protein] + 3 S-adenosyl-L-homocysteine + 3 H(+) (RHEA:54192)

UniProt features (40 total): helix 14, strand 10, sequence variant 5, binding site 4, turn 4, chain 1, modified residue 1, splice variant 1

Structure

Experimental structures (PDB)

1 structures.

PDB	Method	Resolution (Å)
8FZB	X-RAY DIFFRACTION	3.35

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q96G04-F1	92.31	0.81

Functional residue map

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

Ligand- & substrate-binding residues (4): 139; 165–167; 228; 247

Post-translational modifications (1): 1

Function

Pathways and Gene Ontology

Reactome pathways

1 pathways

ID	Pathway
R-HSA-8876725	Protein methylation

MSigDB gene sets: 60 (showing top): BUYTAERT_PHOTODYNAMIC_THERAPY_STRESS_DN, CCAWYNNGAAR_UNKNOWN, BHATI_G2M_ARREST_BY_2METHOXYESTRADIOL_DN, GOBP_PEPTIDYL_LYSINE_MODIFICATION, GATA6_01, GOBP_PEPTIDYL_LYSINE_TRIMETHYLATION, GOBP_METHYLATION, IVANOVA_HEMATOPOIESIS_EARLY_PROGENITOR, GOBP_PEPTIDYL_LYSINE_METHYLATION, GOMF_N_METHYLTRANSFERASE_ACTIVITY, GOMF_PROTEIN_METHYLTRANSFERASE_ACTIVITY, GOMF_S_ADENOSYLMETHIONINE_DEPENDENT_METHYLTRANSFERASE_ACTIVITY, GOMF_TRANSFERASE_ACTIVITY_TRANSFERRING_ONE_CARBON_GROUPS, GOMF_LYSINE_N_METHYLTRANSFERASE_ACTIVITY, REACTOME_POST_TRANSLATIONAL_PROTEIN_MODIFICATION

GO Biological Process (2): peptidyl-lysine trimethylation (GO:0018023), methylation (GO:0032259)

GO Molecular Function (4): protein-lysine N-methyltransferase activity (GO:0016279), protein binding (GO:0005515), methyltransferase activity (GO:0008168), transferase activity (GO:0016740)

GO Cellular Component (3): cytoplasm (GO:0005737), cytosol (GO:0005829), protein-containing complex (GO:0032991)

Reactome top-level categories

Rollup of top-1 pathways:

Category	Pathways
Post-translational protein modification	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	2
peptidyl-lysine methylation	1
metabolic process	1
protein methyltransferase activity	1
lysine N-methyltransferase activity	1
binding	1
transferase activity, transferring one-carbon groups	1
catalytic activity	1
intracellular anatomical structure	1
cytoplasm	1
cellular_component	1

Protein interactions and networks

STRING

366 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
EEF2KMT	EEF1AKMT2	Q5JPI9	806
EEF2KMT	VCPKMT	Q9H867	699
EEF2KMT	METTL21A	Q8WXB1	674
EEF2KMT	ETFBKMT	Q8IXQ9	651
EEF2KMT	METTL22	Q9BUU2	648
EEF2KMT	METTL21C	Q5VZV1	622
EEF2KMT	EEF1AKMT1	Q8WVE0	613
EEF2KMT	KIN	O60870	609
EEF2KMT	METTL18	O95568	601
EEF2KMT	METTL23	Q86XA0	580
EEF2KMT	ANKRD62	A6NC57	563
EEF2KMT	CAMKMT	Q7Z624	538
EEF2KMT	MTMR12	Q9C0I1	524
EEF2KMT	EEF2	P13639	485
EEF2KMT	ANTKMT	Q9BQD7	464

IntAct

49 interactions, top by confidence:

A	B	Type	Score
EEF2KMT	PRTFDC1	psi-mi:“MI:0915”(physical association)	0.830
PRTFDC1	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.830
EEF2KMT	ZNF620	psi-mi:“MI:0915”(physical association)	0.560
EEF2KMT	NR0B1	psi-mi:“MI:0915”(physical association)	0.560
EEF2KMT	PICK1	psi-mi:“MI:0915”(physical association)	0.560
PRKAR1B	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
LNX1	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
KIAA1328	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
IFT88	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
ZNF213	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
TRIP13	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
ZNF620	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
HPRT1	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
NR0B1	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
ZNF35	EEF2KMT	psi-mi:“MI:0915”(physical association)	0.560
EEF2KMT	BCAT2	psi-mi:“MI:0914”(association)	0.530
RFFL	TUSC2	psi-mi:“MI:0914”(association)	0.530
PARL	H2AC21	psi-mi:“MI:0914”(association)	0.350
PARL	CCDC92	psi-mi:“MI:0914”(association)	0.350
EEF2KMT	FAM86B2	psi-mi:“MI:0914”(association)	0.350
EEF2KMT	PRTFDC1	psi-mi:“MI:0915”(physical association)	0.000

BioGRID (40): EEF2 (Affinity Capture-MS), EFT1 (Biochemical Activity), EFT2 (Biochemical Activity), EEF2 (Biochemical Activity), EEF2KMT (Two-hybrid), EEF2 (Affinity Capture-MS), BCAT2 (Affinity Capture-MS), ECI2 (Affinity Capture-MS), GTPBP1 (Affinity Capture-MS), USP15 (Affinity Capture-MS), GALC (Affinity Capture-MS), EEF2KMT (Affinity Capture-MS), EEF2KMT (Two-hybrid), EEF2KMT (Affinity Capture-MS), BCAT2 (Affinity Capture-MS)

ESM2 similar proteins: A2AA28, A4FV42, A4IGU3, A5PK19, A6QP81, A7MBI7, D3YWP0, F6PHZ6, O88587, P0C5J1, P13439, P17256, P21964, P22734, P55345, Q03426, Q14CH1, Q1JPJ9, Q28IN4, Q3TY86, Q3UZW7, Q497B8, Q4JIJ2, Q569C4, Q5BLD8, Q5E9T8, Q5H879, Q5RE14, Q5VZV1, Q6DJF8, Q6GQ33, Q6P3E7, Q8BLU2, Q8BNV1, Q8C1A3, Q8C436, Q8CDZ2, Q8N6R0, Q8N7N1, Q8WXB1

Diamond homologs: A6NEL3, P0C5J1, Q1JPJ9, Q3UZW7, Q8N7N1, Q96G04, Q9NVL1, A4FV42, A4FV98, A4IGU3, A6NDL7, A6QP81, A7IQW5, D3YWP0, F4JNX3, O14118, P38347, P40389, Q28IN4, Q58DC7, Q5BLD8, Q5VZV1, Q8BLU2, Q8C436, Q8CDZ2, Q8R1C6, Q8WXB1, Q96AZ1, Q9CQL0, Q9H867

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	1
Likely pathogenic	0
Uncertain significance	84
Likely benign	20
Benign	1

Top pathogenic / likely-pathogenic (1)

Variant ID	HGVS	Classification
3243497	NC_000016.9:g.(?5132540)(5134882_?)del	Pathogenic

SpliceAI

1333 predictions. Top by Δscore:

Variant	Effect	Δscore
16:5084746:GCAG:G	acceptor_loss	1.0000
16:5084748:A:AG	acceptor_gain	1.0000
16:5084748:AGAT:A	acceptor_gain	1.0000
16:5084749:G:GT	acceptor_gain	1.0000
16:5084749:GA:G	acceptor_gain	1.0000
16:5084749:GAT:G	acceptor_gain	1.0000
16:5084749:GATG:G	acceptor_gain	1.0000
16:5084749:GATGC:G	acceptor_gain	1.0000
16:5085733:C:CC	acceptor_gain	1.0000
16:5089102:CTCA:C	donor_loss	1.0000
16:5089103:TCACC:T	donor_loss	1.0000
16:5089104:CAC:C	donor_loss	1.0000
16:5089106:C:A	donor_loss	1.0000
16:5089106:CCTAG:C	donor_gain	1.0000
16:5089110:G:C	donor_gain	1.0000
16:5089254:CAT:C	acceptor_gain	1.0000
16:5089257:C:CC	acceptor_gain	1.0000
16:5090561:GAGGG:G	acceptor_gain	1.0000
16:5090563:GGG:G	acceptor_gain	1.0000
16:5090563:GGGC:G	acceptor_loss	1.0000
16:5090564:GG:G	acceptor_gain	1.0000
16:5090565:GC:G	acceptor_loss	1.0000
16:5090566:C:CA	acceptor_loss	1.0000
16:5090566:C:CC	acceptor_gain	1.0000
16:5090572:C:CT	acceptor_gain	1.0000
16:5090573:A:T	acceptor_gain	1.0000
16:5091892:CGTG:C	acceptor_gain	1.0000
16:5091896:C:CC	acceptor_gain	1.0000
16:5093561:CAGT:C	acceptor_gain	1.0000
16:5095513:CT:C	acceptor_gain	1.0000

AlphaMissense

2114 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
16:5090265:G:C	S187R	0.985
16:5090265:G:T	S187R	0.985
16:5090267:T:G	S187R	0.985
16:5097680:G:C	F20L	0.982
16:5097680:G:T	F20L	0.982
16:5097682:A:G	F20L	0.982
16:5090328:A:C	S166R	0.981
16:5090328:A:T	S166R	0.981
16:5090330:T:G	S166R	0.981
16:5093494:A:G	L77P	0.974
16:5093505:A:C	F73L	0.974
16:5093505:A:T	F73L	0.974
16:5093507:A:G	F73L	0.974
16:5090344:A:T	V161D	0.972
16:5097649:A:G	W31R	0.970
16:5097649:A:T	W31R	0.970
16:5090095:A:T	V244D	0.966
16:5090493:A:G	W139R	0.965
16:5090493:A:T	W139R	0.965
16:5093506:A:G	F73S	0.965
16:5090214:A:C	N204K	0.955
16:5090214:A:T	N204K	0.955
16:5091803:G:C	S111R	0.954
16:5091803:G:T	S111R	0.954
16:5091805:T:G	S111R	0.954
16:5090491:C:A	W139C	0.953
16:5090491:C:G	W139C	0.953
16:5090144:A:G	W228R	0.950
16:5090144:A:T	W228R	0.950
16:5090276:A:C	Y184D	0.950

dbSNP variants (sampled 300 via entrez): RS1000071413 (16:5094815 A>G), RS1000082838 (16:5098815 A>G), RS1000237447 (16:5085833 T>G), RS1000952792 (16:5087531 G>C), RS1001005100 (16:5087653 C>G,T), RS1001195121 (16:5094162 G>A), RS1001249458 (16:5098601 G>A,C), RS1001362585 (16:5098467 G>A,T), RS1001646513 (16:5094373 C>T), RS1002305270 (16:5090638 C>A,T), RS1002582119 (16:5087278 G>A), RS1002630817 (16:5089826 G>A), RS1003192978 (16:5092279 T>C), RS1003265713 (16:5093156 G>A), RS1003853070 (16:5089512 G>A,T)

Disease associations

OMIM: gene MIM:615263 | disease phenotypes: MIM:608540

GenCC curated gene-disease

Mondo (2): ALG1-congenital disorder of glycosylation (MONDO:0012052), focal segmental glomerulosclerosis (MONDO:0100313)

Orphanet (1): ALG1-CDG (Orphanet:79327)

HPO phenotypes

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

GWAS associations

5 associations (top):

Study	Trait	p-value
GCST001521_36	Subcutaneous adipose tissue	9.000000e-06
GCST002529_1	Glaucoma	1.000000e-08
GCST002529_8	Glaucoma	4.000000e-08
GCST009391_393	Metabolite levels	8.000000e-06
GCST009391_822	Metabolite levels	5.000000e-06

EFO canonical traits (2, from GWAS)

EFO ID	Trait name
EFO:0010437	triacylglycerol 58:10 measurement
EFO:0010398	sphingomyelin 24:1 measurement

MeSH disease descriptors (2)

Descriptor	Name	Tree numbers
D005923	Glomerulosclerosis, Focal Segmental	C12.050.351.968.419.570.363.640; C12.200.777.419.570.363.660; C12.950.419.570.363.640
C535749	Congenital disorder of glycosylation type 1K (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

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

CTD chemical–gene interactions

26 total (human), top 26 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
sodium arsenite	decreases expression, increases expression	2
Tobacco Smoke Pollution	decreases expression, increases expression	2
Tretinoin	decreases expression	2
Cadmium Chloride	decreases expression	2
GSK-J4	decreases expression	1
dicrotophos	increases expression	1
triphenyl phosphate	affects expression	1
bisphenol A	decreases methylation, increases expression	1
tris(2-butoxyethyl) phosphate	affects expression	1
di-n-butylphosphoric acid	affects expression	1
cylindrospermopsin	increases expression	1
perfluoro-n-nonanoic acid	increases expression	1
abrine	decreases expression	1
jinfukang	increases expression	1
NSC 689534	affects binding, decreases expression	1
Benzo(a)pyrene	increases methylation	1
Copper	affects binding, decreases expression	1
Dichlorodiphenyl Dichloroethylene	increases expression	1
Estradiol	increases expression	1
Silicon Dioxide	increases expression	1
Smoke	decreases expression	1
Thiram	decreases expression	1
Urethane	decreases expression	1
Valproic Acid	increases expression	1
Gold Compounds	increases expression	1
Copper Sulfate	decreases expression	1

Clinical trials (associated diseases)

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

Trial	Phase	Status	Title
NCT01129557	PHASE4	TERMINATED	Aldosterone Breakthrough During Diovan, Tekturna, and Combination Therapy in Patients With Proteinuric Kidney Disease
NCT02399462	PHASE4	WITHDRAWN	Acthar for Treatment of Post-transplant FSGS
NCT02585804	PHASE4	COMPLETED	Treating to Reduce Albuminuria and Normalize Hemodynamic Function in Focal ScLerosis With dApagliflozin Trial Effects
NCT02633046	PHASE4	COMPLETED	Acthar for Treatment-Resistant or Treatment-Intolerant Proteinuria
NCT07219121	PHASE4	RECRUITING	Sparsentan in Posttransplant Immunoglobulin A Nephropathy or Focal Segmental Glomerulosclerosis
NCT01164098	PHASE3	TERMINATED	Rituximab to Prevent Recurrence of Proteinuria
NCT02683889	PHASE3	COMPLETED	Use of Acthar in Patients With FSGS That Will be Undergoing Renal Transplantation
NCT03298698	PHASE3	UNKNOWN	Efficacy of Rituximab in Comparison to Continued Corticosteroid Treatment in Idiopathic Nephrotic Syndrome
NCT03493685	PHASE3	COMPLETED	Study of Sparsentan in Patients With Primary Focal Segmental Glomerulosclerosis (FSGS)
NCT05183646	PHASE3	RECRUITING	A Study of the Efficacy and Safety of DMX-200 in Patients With FSGS Who Are Receiving an ARB
NCT07220083	PHASE3	RECRUITING	A Study to Find Out if BI 764198 Helps Adults and Adolescents With a Kidney Condition Called Focal Segmental Glomerulosclerosis (FSGS)
NCT00550342	PHASE2	WITHDRAWN	Rituximab Treatment of Focal Segmental Glomerulosclerosis
NCT00814255	PHASE2	COMPLETED	Novel Therapies for Resistant FSGS (FONTII): Phase II Clinical Trial
NCT01613118	PHASE2	COMPLETED	Randomized, Double-Blind, Safety and Efficacy Study of RE-021 (Sparsentan) in Focal Segmental Glomerulosclerosis
NCT02592798	PHASE2	COMPLETED	Pilot Study to Evaluate the Safety and Efficacy of Abatacept in Adults and Children 6 Years and Older With Excessive Loss of Protein in the Urine Due to Either Focal Segmental Glomerulosclerosis (FSGS) or Minimal Change Disease (MCD)
NCT03366337	PHASE2	COMPLETED	A Phase 2 Trial of the Safety and Efficacy of Bardoxolone Methyl in Patients With Rare Chronic Kidney Diseases - PHOENIX
NCT03448692	PHASE2	TERMINATED	A Study to Evaluate PF-06730512 in Adults With Focal Segmental Glomerulosclerosis (FSGS)
NCT03536754	PHASE2	COMPLETED	A Study of CCX140-B in Subjects With FSGS
NCT03598036	PHASE2	TERMINATED	Dose-Exploration Evaluating the Efficacy and Safety of Voclosporin in Subjects With Focal Segmental Glomerulosclerosis
NCT03649152	PHASE2	COMPLETED	Safety and Effectiveness of Propagermanium in Focal Segmental Glomerulosclerosis Participants Receiving Irbesartan
NCT03703908	PHASE2	TERMINATED	A Study of CCX140-B in Subjects With Primary FSGS and Nephrotic Syndrome
NCT04009668	PHASE2	COMPLETED	Tumor Necrosis Factor Inhibition in Focal Segmental Glomerulosclerosis and Treatment Resistant Minimal Change Disease
NCT04573920	PHASE2	ACTIVE_NOT_RECRUITING	Atrasentan in Patients With Proteinuric Glomerular Diseases
NCT05003986	PHASE2	RECRUITING	Study of Sparsentan Treatment in Pediatrics With Proteinuric Glomerular Diseases
NCT05267262	PHASE2	COMPLETED	Study to Evaluate R3R01 in Patients With Alport Syndrome and Patients With Focal Segmental Glomerulosclerosis
NCT05441826	PHASE2	TERMINATED	Efficacy and Safety of VB119 in Subjects With Minimal Change Disease (MCD) and Focal Segmental Glomerulosclerosis (FSGS)
NCT06500702	PHASE2	RECRUITING	A Study to Evaluate the Efficacy and Safety of Frexalimab, Brivekimig, or Rilzabrutinib in Participants Aged 16 to 75 Years With Primary Focal Segmental Glomerulosclerosis or Minimal Change Disease
NCT06664814	PHASE2	RECRUITING	An Open-Label Phase 2 Study of N-Acetyl-D-Mannosamine (ManNAc) in Subjects With Primary Focal Segmental Glomerulosclerosis
NCT06983028	PHASE2	RECRUITING	Atacicept in Multiple Glomerular Diseases
NCT07268638	PHASE2	RECRUITING	A Study of Praliciguat in Participants With Focal Segmental Glomerulosclerosis (FSGS)
NCT07614477	PHASE2	RECRUITING	Evaluate the Efficacy, Safety, Pharmacokinetics, and Pharmacodynamics of EVER001 in Participants With Selected Proteinuric Glomerular Diseases
NCT00464321	PHASE1	COMPLETED	Safety Study of GC1008 in Patients With Focal Segmental Glomerulosclerosis (FSGS) of Single Doses of GC1008 in Patients With Treatment Resistant Idiopathic FSGS
NCT00782561	PHASE1	TERMINATED	Safety and Pharmacokinetics of FG-3019 in Adolescents and Adults With Focal Segmental Glomerulosclerosis (FSGS)
NCT00816478	PHASE1	TERMINATED	Effect of Oral Galactose on Focal Segmental Glomerulosclerosis (FSGS) Permeability Factor
NCT00816504	PHASE1	WITHDRAWN	Effect of Galactose on Permeblity Factor in Patients With FSGS and CKD Stage 5
NCT02382874	PHASE1	UNKNOWN	Allogenic AD-MSC Transplantation in Idiopathic Nephrotic Syndrome (Focal Segmental Glomerulosclerosis)
NCT02693366	PHASE1	COMPLETED	Stem Cell Therapy for Patients With Focal Segmental Glomerulosclerosis
NCT05942625	PHASE1	RECRUITING	A First in Human Study to Evaluate Safety, Tolerability, Pharmacology of HS-10390 in Healthy Subjects
NCT05955872	PHASE1	COMPLETED	A Study Evaluating the Relative Bioavailability and Food Effect of a Tablet Formulation of VX-147
NCT06529796	PHASE1	COMPLETED	Evaluation of the Pharmacokinetics and Safety of Inaxaplin in Participants With Mild or Moderate Hepatic Impairment