TPRKB

Summary

TPRKB (TP53RK binding protein, HGNC:24259) is a protein-coding gene on chromosome 2p13.1, encoding EKC/KEOPS complex subunit TPRKB (Q9Y3C4). Component of the EKC/KEOPS complex that is required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in tRNAs that read codons beginning with adenine.

Enables protein kinase binding activity. Involved in tRNA threonylcarbamoyladenosine modification. Located in cytosol and nucleus. Part of EKC/KEOPS complex. Implicated in Galloway-Mowat syndrome 5.

Source: NCBI Gene 51002 — RefSeq curated summary.

At a glance

• Gene–disease (curated): Galloway-Mowat syndrome 5 (Strong, GenCC) — +1 more curated relationship
• Clinical variants (ClinVar): 69 total — 1 pathogenic, 1 likely-pathogenic
• Phenotypes (HPO): 47
• Druggable target: yes
• MANE Select transcript: NM_016058

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:24259
Approved symbol	TPRKB
Name	TP53RK binding protein
Location	2p13.1
Locus type	gene with protein product
Status	Approved
Aliases	CGI-121, CGI121
Ensembl gene	ENSG00000144034
Ensembl biotype	protein_coding
OMIM	608680
Entrez	51002

Gene structure

Transcript identifiers

Ensembl transcripts: 49 — 42 protein_coding, 5 protein_coding_CDS_not_defined, 2 retained_intron

ENST00000272424, ENST00000318190, ENST00000409716, ENST00000462166, ENST00000463231, ENST00000470012, ENST00000484969, ENST00000485758, ENST00000489476, ENST00000497464, ENST00000904803, ENST00000904804, ENST00000904805, ENST00000904806, ENST00000904807, ENST00000904808, ENST00000904809, ENST00000904810, ENST00000904811, ENST00000939335, ENST00000939336, ENST00000939337, ENST00000939338, ENST00000939339, ENST00000939340, ENST00000939341, ENST00000939342, ENST00000939343, ENST00000939344, ENST00000939345, ENST00000939346, ENST00000939347, ENST00000939348, ENST00000939349, ENST00000939350, ENST00000939351, ENST00000939352, ENST00000939353, ENST00000939354, ENST00000939355, ENST00000939356, ENST00000939357, ENST00000939358, ENST00000939359, ENST00000939360, ENST00000939361, ENST00000939362, ENST00000939363, ENST00000965314

RefSeq mRNA: 8 — MANE Select: NM_016058 NM_001330386, NM_001330387, NM_001330388, NM_001330389, NM_001330390, NM_001330391, NM_001330392, NM_016058

CCDS: CCDS1927, CCDS82471

Canonical transcript exons

ENST00000272424 — 5 exons

Exon	Start	End
ENSE00000963339	73729873	73730029
ENSE00003463429	73732163	73732285
ENSE00003638996	73734429	73734591
ENSE00003653356	73730560	73730736
ENSE00003894433	73737302	73737345

Expression profiles

Bgee: expression breadth ubiquitous, 284 present calls, max score 97.38.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 24.0825 / max 448.9475, expressed in 1802 samples.

FANTOM5 promoters (3 alternative TSS)

Promoter ID	TPM avg	Samples expressed
29146	22.9887	1800
202237	0.5721	363
29145	0.5217	296

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
right uterine tube	UBERON:0001302	97.38	gold quality
ganglionic eminence	UBERON:0004023	97.07	gold quality
right testis	UBERON:0004534	95.86	gold quality
monocyte	CL:0000576	95.82	gold quality
left testis	UBERON:0004533	95.82	gold quality
mononuclear cell	CL:0000842	95.78	gold quality
embryo	UBERON:0000922	95.68	gold quality
leukocyte	CL:0000738	95.43	gold quality
calcaneal tendon	UBERON:0003701	95.33	gold quality
ventricular zone	UBERON:0003053	95.29	gold quality
cortical plate	UBERON:0005343	95.27	gold quality
gastrocnemius	UBERON:0001388	95.26	gold quality
rectum	UBERON:0001052	95.20	gold quality
muscle of leg	UBERON:0001383	95.19	gold quality
left ovary	UBERON:0002119	95.16	gold quality
right ovary	UBERON:0002118	95.10	gold quality
testis	UBERON:0000473	94.83	gold quality
C1 segment of cervical spinal cord	UBERON:0006469	94.71	gold quality
muscle organ	UBERON:0001630	94.59	gold quality
islet of Langerhans	UBERON:0000006	94.57	gold quality
body of pancreas	UBERON:0001150	94.52	gold quality
hindlimb stylopod muscle	UBERON:0004252	94.39	gold quality
left uterine tube	UBERON:0001303	94.32	gold quality
biceps brachii	UBERON:0001507	94.27	gold quality
lower esophagus mucosa	UBERON:0035834	94.22	gold quality
cerebellar hemisphere	UBERON:0002245	94.07	gold quality
ovary	UBERON:0000992	94.02	gold quality
cerebellar cortex	UBERON:0002129	94.00	gold quality
triceps brachii	UBERON:0001509	93.81	gold quality
body of uterus	UBERON:0009853	93.80	gold quality

Single-cell (SCXA)

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

Experiment	Marker?	Max mean expression
E-HCAD-13	yes	20.52
E-ANND-3	no	0.00

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

7 targeting TPRKB, 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-651-3P	99.94	73.48	5177
HSA-MIR-627-3P	99.90	71.42	3316
HSA-MIR-5003-3P	99.85	69.29	2517
HSA-MIR-4679	99.76	69.19	1229
HSA-MIR-5700	99.64	69.88	2280
HSA-MIR-2053	99.57	69.15	1635
HSA-MIR-155-5P	99.35	70.16	1509

Literature-anchored findings (GeneRIF, showing 2)

• Crystal structure of the human PRPK-TPRKB complex. (PMID:33547416)
• METTL5 enhances the mRNA stability of TPRKB through m[6]A modification to facilitate the aggressive phenotypes of hepatocellular carcinoma cell. (PMID:39182664)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
danio_rerio	tprkb	ENSDARG00000014941
mus_musculus	Tprkb	ENSMUSG00000054226
rattus_norvegicus	Tprkb	ENSRNOG00000050645
caenorhabditis_elegans		WBGENE00020994

Protein

Protein identifiers

EKC/KEOPS complex subunit TPRKB — Q9Y3C4 (reviewed: Q9Y3C4)

Alternative names: PRPK-binding protein, TP53RK-binding protein

All UniProt accessions (1): Q9Y3C4

UniProt curated annotations — full annotation on UniProt →

Function. Component of the EKC/KEOPS complex that is required for the formation of a threonylcarbamoyl group on adenosine at position 37 (t(6)A37) in tRNAs that read codons beginning with adenine. The complex is probably involved in the transfer of the threonylcarbamoyl moiety of threonylcarbamoyl-AMP (TC-AMP) to the N6 group of A37. TPRKB acts as an allosteric effector that regulates the t(6)A activity of the complex. TPRKB is not required for tRNA modification.

Subunit / interactions. Component of the EKC/KEOPS complex composed of at least GON7, TP53RK, TPRKB, OSGEP and LAGE3; the whole complex dimerizes. Interacts with TP53RK/PRPK.

Subcellular location. Cytoplasm. Cytosol. Nucleus.

Tissue specificity. Widely expressed.

Disease relevance. Galloway-Mowat syndrome 5 (GAMOS5) [MIM:617731] A form of Galloway-Mowat syndrome, a severe renal-neurological disease characterized by early-onset nephrotic syndrome associated with microcephaly, central nervous system abnormalities, developmental delays, and a propensity for seizures. Brain anomalies include gyration defects ranging from lissencephaly to pachygyria and polymicrogyria, and cerebellar hypoplasia. Most patients show facial dysmorphism characterized by a small, narrow forehead, large/floppy ears, deep-set eyes, hypertelorism and micrognathia. Additional variable features are visual impairment and arachnodactyly. Most patients die in early childhood. The disease is caused by variants affecting the gene represented in this entry.

Similarity. Belongs to the CGI121/TPRKB family.

Isoforms (3)

UniProt ID	Names	Canonical?
Q9Y3C4-1	1	yes
Q9Y3C4-2	2, S1
Q9Y3C4-3	3, L1

RefSeq proteins (8): NP_001317315, NP_001317316, NP_001317317, NP_001317318, NP_001317319, NP_001317320, NP_001317321, NP_057142* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR013926	CGI121/TPRKB	Family
IPR036504	CGI121/TPRKB_sf	Homologous_superfamily

Pfam: PF08617

UniProt features (26 total): helix 12, strand 8, splice variant 2, sequence variant 2, chain 1, sequence conflict 1

Structure

Experimental structures (PDB)

7 structures.

PDB	Method	Resolution (Å)
7SZC	X-RAY DIFFRACTION	1.71
7SZA	X-RAY DIFFRACTION	1.9
7SZB	X-RAY DIFFRACTION	2.02
7SZD	X-RAY DIFFRACTION	2.05
3ENP	X-RAY DIFFRACTION	2.48
6WQX	X-RAY DIFFRACTION	2.53
9FL9	ELECTRON MICROSCOPY	3.74

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q9Y3C4-F1	95.47	0.91

Function

Pathways and Gene Ontology

Reactome pathways

1 pathways

ID	Pathway
R-HSA-6782315	tRNA modification in the nucleus and cytosol

MSigDB gene sets: 219 (showing top): MODULE_151, GOBP_TRNA_METABOLIC_PROCESS, GRAESSMANN_APOPTOSIS_BY_SERUM_DEPRIVATION_UP, CHIANG_LIVER_CANCER_SUBCLASS_UNANNOTATED_DN, GOBP_TRANSLATION, WEI_MYCN_TARGETS_WITH_E_BOX, GOBP_RNA_MODIFICATION, SHETH_LIVER_CANCER_VS_TXNIP_LOSS_PAM4, GARCIA_TARGETS_OF_FLI1_AND_DAX1_DN, BASAKI_YBX1_TARGETS_UP, GOBP_TRNA_THREONYLCARBAMOYLADENOSINE_METABOLIC_PROCESS, GOBP_TRNA_PROCESSING, MODULE_114, REACTOME_METABOLISM_OF_RNA, GOBP_TRNA_MODIFICATION

GO Biological Process (2): tRNA threonylcarbamoyladenosine modification (GO:0002949), tRNA processing (GO:0008033)

GO Molecular Function (2): protein kinase binding (GO:0019901), protein binding (GO:0005515)

GO Cellular Component (4): EKC/KEOPS complex (GO:0000408), nucleus (GO:0005634), cytoplasm (GO:0005737), cytosol (GO:0005829)

Reactome top-level categories

Rollup of top-1 pathways:

Category	Pathways
tRNA processing	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	2
tRNA modification	1
RNA processing	1
tRNA metabolic process	1
kinase binding	1
binding	1
transferase complex	1
intracellular membrane-bounded organelle	1
intracellular anatomical structure	1
cytoplasm	1

Protein interactions and networks

STRING

1286 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
TPRKB	TP53RK	Q96S44	999
TPRKB	OSGEP	Q9NPF4	998
TPRKB	LAGE3	Q14657	998
TPRKB	GON7	Q9BXV9	991
TPRKB	YRDC	Q86U90	874
TPRKB	OSGEPL1	Q9H4B0	872
TPRKB	BUD31	P41223	526
TPRKB	TP53	P04637	492
TPRKB	POLR1B	Q9H9Y6	398
TPRKB	GNL2	Q13823	373
TPRKB	POLR1D	P0DPB6	370
TPRKB	RAD52	P43351	367
TPRKB	SH2D2A	Q9NP31	358
TPRKB	DDX5	P17844	353
TPRKB	HTRA3	P83110	353
TPRKB	TCP1	P17987	353
TPRKB	F6RGN5	F6RGN5	353

IntAct

41 interactions, top by confidence:

A	B	Type	Score
UBA5	GABARAPL2	psi-mi:“MI:0914”(association)	0.950
CPSF6	NUDT21	psi-mi:“MI:0914”(association)	0.890
GON7	LAGE3	psi-mi:“MI:0915”(physical association)	0.880
GON7	LAGE3	psi-mi:“MI:0914”(association)	0.880
TPRKB	TP53RK	psi-mi:“MI:0915”(physical association)	0.850
TP53RK	TPRKB	psi-mi:“MI:0915”(physical association)	0.850
TP53RK	GON7	psi-mi:“MI:0914”(association)	0.820
TP53RK	NUP43	psi-mi:“MI:0914”(association)	0.730
NUP43	NUP98	psi-mi:“MI:0914”(association)	0.640
TPRKB	TRIM27	psi-mi:“MI:0915”(physical association)	0.560
TRIM27	TPRKB	psi-mi:“MI:0915”(physical association)	0.560
POP7	RPP40	psi-mi:“MI:0914”(association)	0.530
NUP43	KIF5B	psi-mi:“MI:0914”(association)	0.530
ANO4	ANO6	psi-mi:“MI:0914”(association)	0.530
GORASP1	PPP6R2	psi-mi:“MI:0914”(association)	0.530
LAGE3	CTSA	psi-mi:“MI:0914”(association)	0.530
MBIP	TADA2A	psi-mi:“MI:0914”(association)	0.530
TPRKB	H1-5	psi-mi:“MI:0915”(physical association)	0.400
TPRKB	E6	psi-mi:“MI:0915”(physical association)	0.370
Osgep	RPSA	psi-mi:“MI:0914”(association)	0.350
TP53RK	CLTB	psi-mi:“MI:0914”(association)	0.350
POLR3E	BDP1	psi-mi:“MI:0914”(association)	0.350
MBIP	CIBAR1	psi-mi:“MI:0914”(association)	0.350
LAGE3	HYKK	psi-mi:“MI:0914”(association)	0.350
OSGEP	HYKK	psi-mi:“MI:0914”(association)	0.350
MSH5	GET1	psi-mi:“MI:0914”(association)	0.350
GORASP1	RTCA	psi-mi:“MI:0914”(association)	0.350

BioGRID (99): TPRKB (Two-hybrid), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TP53RK (Two-hybrid), TPRKB (Affinity Capture-Western), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS), TPRKB (Affinity Capture-MS)

ESM2 similar proteins: A1RWX0, A2SFM8, A6MMC5, A6MMU7, B0BSZ8, B1I710, B2IRP8, B2XWK7, B5E6S0, B8ZM41, C1C8N4, C1CFL4, C3MUV8, C3N5H2, C4K473, C4KH38, O01374, O33510, O33543, O33544, O33575, O78442, P06942, P18611, P30879, P32285, P35976, P52201, Q04JC5, Q09MH5, Q0G9L6, Q10735, Q21U90, Q2MI39, Q2MIE3, Q2VED6, Q32RZ2, Q3C1N3, Q491W4, Q5BB99

Diamond homologs: A1CEY5, F1QZ15, Q03705, Q0C9R3, Q0UEM3, Q1DYR7, Q4ID21, Q4WI37, Q5PQR8, Q6BP94, Q6C7C9, Q6CT72, Q75D21, Q7SHG9, Q8QZZ7, Q9P7J3, Q9Y3C4, P0CM64, P0CM65, Q4P144, Q5A519, Q6FV72

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	1
Likely pathogenic	1
Uncertain significance	42
Likely benign	8
Benign	9

Top pathogenic / likely-pathogenic (2)

Variant ID	HGVS	Classification
444885	NM_016058.5(TPRKB):c.446A>G (p.Tyr149Cys)	Pathogenic
3065453	NM_016058.5(TPRKB):c.445T>A (p.Tyr149Asn)	Likely pathogenic

SpliceAI

0 predictions. Top by Δscore:

AlphaMissense

1141 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
2:73732246:C:G	A61P	0.973
2:73729955:T:A	K172N	0.969
2:73729955:T:G	K172N	0.969
2:73732179:A:G	L83P	0.963
2:73730713:A:C	F96L	0.962
2:73730713:A:T	F96L	0.962
2:73730715:A:G	F96L	0.962
2:73732257:G:T	A57E	0.962
2:73729956:T:A	K172I	0.959
2:73732194:T:A	E78V	0.956
2:73732245:G:T	A61E	0.952
2:73732266:A:T	I54K	0.938
2:73732193:T:A	E78D	0.931
2:73732193:T:G	E78D	0.931
2:73730727:C:G	A92P	0.930
2:73730684:A:G	L106P	0.925
2:73730711:C:T	G97D	0.924
2:73730735:A:G	I89T	0.923
2:73732212:G:A	T72I	0.921
2:73730723:A:G	L93S	0.916
2:73732266:A:C	I54R	0.914
2:73730619:C:G	G128R	0.912
2:73732247:T:A	K60N	0.910
2:73732247:T:G	K60N	0.910
2:73732255:C:G	A58P	0.910
2:73734510:A:C	F20L	0.910
2:73734510:A:T	F20L	0.910
2:73734512:A:G	F20L	0.910
2:73729967:T:A	R168S	0.909
2:73729967:T:G	R168S	0.909

dbSNP variants (sampled 300 via entrez): RS1000474066 (2:73737689 G>A), RS1000525991 (2:73737938 C>A,T), RS1000983296 (2:73731853 G>A), RS1000993173 (2:73731586 C>G), RS1001546679 (2:73737421 T>A,C,G), RS1001693284 (2:73731140 T>C), RS1001742641 (2:73730909 C>A,G), RS1001874597 (2:73732017 C>A,T), RS1001886646 (2:73737616 T>C), RS1002636907 (2:73735452 G>A), RS1002694801 (2:73729455 A>C), RS1002818821 (2:73733567 T>G), RS1002898375 (2:73732463 G>C), RS1002973647 (2:73735728 C>A), RS1003562366 (2:73738975 A>G)

Disease associations

OMIM: gene MIM:608680 | disease phenotypes: MIM:617731

GenCC curated gene-disease

Disease	Classification	Inheritance
Galloway-Mowat syndrome 5	Strong	Autosomal recessive
Galloway-Mowat syndrome	Supportive	Autosomal recessive

Mondo (3): Galloway-Mowat syndrome 5 (MONDO:0033009), nephrotic syndrome (MONDO:0005377), Galloway-Mowat syndrome (MONDO:0009627)

Orphanet (0):

HPO phenotypes

47 total (30 of 47 shown, HPO-id order):

HPO	Term
HP:0000007	Autosomal recessive inheritance
HP:0000093	Proteinuria
HP:0000097	Focal segmental glomerulosclerosis
HP:0000100	Nephrotic syndrome
HP:0000112	Nephropathy
HP:0000164	Abnormality of the dentition
HP:0000252	Microcephaly
HP:0000276	Long face
HP:0000286	Epicanthus
HP:0000303	Mandibular prognathia
HP:0000316	Hypertelorism
HP:0000347	Micrognathia
HP:0000365	Hearing impairment
HP:0000400	Macrotia
HP:0000490	Deeply set eye
HP:0000601	Hypotelorism
HP:0000969	Edema
HP:0001181	Adducted thumb
HP:0001249	Intellectual disability
HP:0001250	Seizure
HP:0001251	Ataxia
HP:0001252	Hypotonia
HP:0001257	Spasticity
HP:0001263	Global developmental delay
HP:0001276	Hypertonia
HP:0001302	Pachygyria
HP:0001511	Intrauterine growth retardation
HP:0001622	Premature birth
HP:0002036	Hiatus hernia
HP:0002119	Ventriculomegaly

GWAS associations

0 associations (top):

MeSH disease descriptors (2)

Descriptor	Name	Tree numbers
D009404	Nephrotic Syndrome	C12.050.351.968.419.630.643; C12.200.777.419.630.643; C12.950.419.630.643
C537548	Galloway Mowat syndrome (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (1): CHEMBL6066493 (SINGLE PROTEIN)

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

ChEMBL bioactivities

2 potent at pChembl≥5 of 4 total, top 2 by pChembl (potency: 10 = 0.1 nM, 6 = 1 µM).

pChembl	Type	Value	Unit	Molecule
6.28	Kd	521	nM	CHEMBL5653589
6.26	ED50	550.3	nM	CHEMBL5653589

PubChem BioAssay actives

1 with measured affinity, of 4 total; 1 most potent distinct compounds. Largely complementary to BindingDB; screening values are coarse (µM, 4 dp), so sub-nM hits tie at the floor.

Compound	Assay	Type	Value	Unit
4-methyl-3-[(2-methyl-6-pyridin-3-ylpyrazolo[3,4-d]pyrimidin-4-yl)amino]-N-[3-(trifluoromethyl)phenyl]benzamide	2149648: Binding affinity to human TPRKB incubated for 45 mins by Kinobead based pull down assay	kd	0.5210	uM

CTD chemical–gene interactions

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

Chemical	Actions (top 5)	PubMed papers
sodium arsenite	decreases expression, affects cotreatment, increases abundance, increases expression	3
bisphenol A	decreases expression, decreases methylation	2
Air Pollutants	increases abundance, increases oxidation, decreases expression, affects cotreatment	2
Arsenic	affects cotreatment, increases abundance, increases expression, decreases expression	2
Valproic Acid	affects expression, increases expression	2
Particulate Matter	decreases expression, increases abundance, affects cotreatment	2
dicrotophos	decreases expression	1
alpha-pinene	increases abundance, affects cotreatment, increases oxidation	1
arsenite	affects binding, increases reaction	1
manganese chloride	affects cotreatment, increases abundance, increases expression	1
methacrylaldehyde	affects cotreatment, increases oxidation, increases abundance	1
di-n-butylphosphoric acid	affects expression	1
CGP 52608	increases reaction, affects binding	1
jinfukang	affects cotreatment, decreases expression	1
Resveratrol	affects cotreatment, increases expression	1
Vorinostat	increases expression	1
Acrolein	affects cotreatment, increases oxidation, increases abundance	1
Benzo(a)pyrene	affects methylation	1
Cadmium	increases abundance, increases expression	1
Cisplatin	affects cotreatment, decreases expression	1
Copper	affects binding, decreases expression	1
Disulfiram	affects binding, decreases expression	1
Estradiol	decreases expression	1
Gasoline	affects cotreatment, decreases expression, increases abundance	1
Manganese	affects cotreatment, increases abundance, increases expression	1
Ozone	affects cotreatment, increases oxidation, increases abundance	1
Plant Extracts	affects cotreatment, increases expression	1
Polycyclic Aromatic Hydrocarbons	affects cotreatment, decreases expression, increases abundance	1
Rotenone	decreases expression	1
Thimerosal	increases expression	1

ChEMBL screening assays

1 unique, capped per target: 1 binding

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL5652690	Binding	Binding affinity to human TPRKB incubated for 45 mins by Kinobead based pull down assay	NVP-BHG712: Effects of Regioisomers on the Affinity and Selectivity toward the EPHrin Family. — ChemMedChem

Clinical trials (associated diseases)

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

Trial	Phase	Status	Title
NCT00308321	PHASE4	UNKNOWN	Long Term Tapering or Standard Steroids for Nephrotic Syndrome
NCT01021540	PHASE4	COMPLETED	Prospective Study Evaluating the Effect of Repository Corticotropin in the Treatment of Various Nephrotic Syndromes
NCT01028287	PHASE4	COMPLETED	Adrenocorticotropic Hormone (ACTH) Treatment of Nephrotic Range Proteinuria in Diabetic Nephropathy (NRDN)
NCT01162005	PHASE4	COMPLETED	Therapeutic Effect of Tacrolimus on Primary Nephrotic Syndrome in Children
NCT01895894	PHASE4	COMPLETED	Mycophenolate Mofetil in Pediatric Steroid Dependent Nephrotic Syndrome
NCT02238418	PHASE4	COMPLETED	Efficacy of Usual Vitamin D Supplementation and Its Impact on Children and Adolescents Calciuria.
NCT02382575	PHASE4	UNKNOWN	Efficacy and Safety of Rituximab to That of Calcineurin Inhibitors in Children With Steroid Resistant Nephrotic Syndrome
NCT02427880	PHASE4	COMPLETED	Role of Acetazolamide and Hydrochlorothiazide Followed by Furosemide in Treating Nephrotic Edema
NCT03210688	PHASE4	COMPLETED	Active Vitamin D And Reduced Dose Prednisolone for Treatment in Minimal Change Nephropathy
NCT03347357	PHASE4	COMPLETED	Pharmacokinetics of Tacrolimus in Children
NCT05696977	PHASE4	UNKNOWN	Effect of Obesity on Cyclosporine Blood Trough Level in Nephrotic Syndrome Patients
NCT05966818	PHASE4	UNKNOWN	Effect of Dapagliflozin in Non-Diabetic Patients With Nephrotic Syndrome.
NCT06026787	PHASE4	COMPLETED	Clinical Value of Adding Dapagliflozin in Patients With Nephrotic Syndrome
NCT00354731	PHASE3	COMPLETED	Efficacy of Pentoxifylline on Primary Nephrotic Syndrome
NCT00615667	PHASE3	COMPLETED	Prospective, Multicenter Study of the Efficacy and Tolerance of Tacrolimus on Refractory Nephrotic Syndrome (RNS)
NCT00981838	PHASE3	COMPLETED	Rituximab in Multirelapsing Minimal Change Disease (MCD) or Focal Segmental Glomerulosclerosis (FSGS)
NCT01197040	PHASE3	COMPLETED	Evaluation of Low Dose Corticosteroids Efficiency, Associated With Myfortic ® in the Treatment of Nephrotic Syndrome
NCT01309477	PHASE3	COMPLETED	The Efficacy and Tolerance of Tacrolimus Sustained-release Capsules on Refractory Nephrotic Syndrome (RNS)
NCT02132195	PHASE3	COMPLETED	Adrenocorticotropic Hormone (ACTH) for Frequently Relapsing and Steroid Dependent Nephrotic Syndrome
NCT02257697	PHASE3	COMPLETED	A Study to Evaluate the Efficacy and Safety of Mizoribine in the Treatment of Refractory Nephrotic Syndrome
NCT02438982	PHASE3	COMPLETED	Efficacy and Safety of Rituximab to That of Calcineurin Inhibitors in Children With Steroid Dependent Nephrotic Syndrome
NCT03141970	PHASE3	COMPLETED	Prednisolone Trial in Children Younger Than 4 Years
NCT03501459	PHASE3	UNKNOWN	Lymphocyte Markers As Predictors Of Responsiveness To Rituximab Among Patients With Idiopathic Nephrotic Syndrome
NCT05079789	PHASE3	TERMINATED	Amiloride in Nephrotic Syndrome
NCT05716880	PHASE3	RECRUITING	Ketoanalogues for Muscle Mass Loss in Nephrotic Syndrome
NCT06635720	PHASE3	ACTIVE_NOT_RECRUITING	REduced-dose Steroid PrOtocol for Childhood Nephrotic SyndromE (RESPONSE)
NCT00001212	PHASE2	COMPLETED	Drug Therapy in Lupus Nephropathy
NCT00001959	PHASE2	COMPLETED	Pirfenidone to Treat Kidney Disease (Focal Segmental Glomerulosclerosis)
NCT00004466	PHASE2	TERMINATED	Pilot Study of Atorvastatin in Children With Chronic Hyperlipidemia Secondary to Nephrotic Syndrome
NCT00004990	PHASE2	COMPLETED	Once-A-Month Steroid Treatment for Patients With Focal Segmental Glomerulosclerosis
NCT00977977	PHASE2	RECRUITING	Rituximab Plus Cyclosporine in Idiopathic Membranous Nephropathy
NCT02394106	PHASE2	TERMINATED	Ofatumumab in Children With Drug Resistant Idiopathic Nephrotic Syndrome
NCT02394119	PHASE2	COMPLETED	Ofatumumab Versus Rituximab in Children With Steroid and Calcineurin Inhibitor Dependent Idiopathic Nephrotic Syndrome
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)
NCT02966717	PHASE2	UNKNOWN	Rituximab Combined With MSCs in the Treatment of PNS (3-4 Stage of CKD)
NCT03004001	PHASE2	TERMINATED	Effect of PCSK9-Antibody (Alirocumab) on Dyslipidemia Secondary to Nephrotic Syndrome
NCT03949855	PHASE2	RECRUITING	Belimumab With Rituximab for Primary Membranous Nephropathy
NCT05599815	PHASE2	WITHDRAWN	Part 1 - A Clinical Trial in Patients With Frequently Relapsing and Steroid-Dependent Nephrotic Syndrome
NCT05704400	PHASE2	UNKNOWN	Efficacy of Anti-CD20 Ab Associated With Anti-CD38 in the Childhood Multidrug Dependent and Resistant Nephrotic Syndrome
NCT06983028	PHASE2	RECRUITING	Atacicept in Multiple Glomerular Diseases