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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PRCC
proline rich mitotic checkpoint control factor
Chromosome 1 · 1q23.1
NCBI Gene: 5546Ensembl: ENSG00000143294.16HGNC: HGNC:9343UniProt: A0A0S2Z456
68PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Oncogene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingregulation of cell cyclenucleusnuclear speckneurodegenerative diseaserenal carcinomadengue diseasepapillary renal cell carcinoma
✦AI Summary

PRCC (proline rich mitotic checkpoint control factor) is a nuclear protein predicted to regulate cell cycle progression through interaction with MAD2L2 [UniProt]. The gene is located on chromosome 1 and localizes to nuclear specks and the nucleoplasm, where it functions in protein binding and cell cycle regulation [GO Annotations]. While the provided abstracts do not directly characterize PRCC's molecular mechanism or primary biochemical function, they do establish PRCC's clinical significance in renal cell carcinoma (RCC). PRCC is notable as a fusion partner in papillary renal cell carcinoma (pRCC), forming the PRCC-TFE3 fusion protein in TFE3 translocation RCC (TFE3 tRCC). The PRCC-TFE3 fusion constitutively activates PRKN expression, leading to PINK1-PRKN-dependent mitophagy that promotes cell survival under mitochondrial oxidative stress and enhances proliferation through decreased mitochondrial ROS formation 1. Additionally, PRCC-TFE3 fusion accelerates mitochondrial turnover via PPARGC1A/PGC1α-NRF1 activation 1. pRCC represents the second most common renal carcinoma subtype with heterogeneous molecular features and prognosis 2, and metastatic pRCC carries particularly poor prognosis compared to other non-clear cell RCC subtypes 3. These findings suggest PRCC's involvement in oncogenic transformation through gene fusion mechanisms.

Sources cited
1
PRCC-TFE3 fusion protein constitutively activates PRKN expression, promoting PINK1-PRKN-dependent mitophagy and cell proliferation; fusion accelerates mitochondrial turnover via PPARGC1A/PGC1α-NRF1
PMID: 33019842
2
pRCC is the second most common renal carcinoma type with heterogeneous molecular subtypes showing different clinical outcomes
PMID: 30507616
3
Advanced pRCC has the poorest prognosis among non-clear cell RCC subtypes
PMID: 40437605
⚠Limited data available — This gene has 3 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.52Moderate
renal carcinomaOpen Targets
0.37Weak
dengue diseaseOpen Targets
0.37Weak
papillary renal cell carcinomaOpen Targets
0.23Weak
hepatocellular carcinomaOpen Targets
0.20Weak
prostate carcinomaOpen Targets
0.20Weak
Renal Cell Carcinoma Associated with Xp11.2 Translocations/TFE3 Gene FusionsOpen Targets
0.20Weak
PEComaOpen Targets
0.19Weak
melanomaOpen Targets
0.19Weak
clear cell renal carcinomaOpen Targets
0.19Weak
head and neck squamous cell carcinomaOpen Targets
0.19Weak
urinary bladder carcinomaOpen Targets
0.19Weak
kidney neoplasmOpen Targets
0.19Weak
acute myeloid leukemiaOpen Targets
0.19Weak
lung adenocarcinomaOpen Targets
0.19Weak
bladder transitional cell carcinomaOpen Targets
0.19Weak
papillary thyroid carcinomaOpen Targets
0.19Weak
oral squamous cell carcinomaOpen Targets
0.19Weak
squamous cell lung carcinomaOpen Targets
0.19Weak
cutaneous melanomaOpen Targets
0.18Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CDK8Shared pathway100%CDKL4Shared pathway100%MRFAP1L2Shared pathway100%CABLES2Shared pathway100%PRR11Shared pathway100%PGCKA1Shared pathway100%
Tissue Expression6 tissues
Brain
100%
Lung
75%
Bone Marrow
69%
Heart
68%
Ovary
68%
Liver
64%
Gene Interaction Network
Click a node to explore
PRCCCDK8CDKL4MRFAP1L2CABLES2PRR11PGCKA1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q92733
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.40Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.24 [0.15–0.40]
RankingsWhere PRCC stands among ~20K protein-coding genes
  • #6,935of 20,598
    Most Researched68
  • #2,032of 17,882
    Most Constrained (LOEUF)0.40 · top quartile
Genes detectedPRCC
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The Cancer Genome Atlas of renal cell carcinoma: findings and clinical implications.
PMID: 31278395
Nat Rev Urol · 2019
1.00
2
A comparison of sunitinib with cabozantinib, crizotinib, and savolitinib for treatment of advanced papillary renal cell carcinoma: a randomised, open-label, phase 2 trial.
PMID: 33592176
Lancet · 2021
0.90
3
Epigenomic charting and functional annotation of risk loci in renal cell carcinoma.
PMID: 36681680
Nat Commun · 2023
0.80
4
Integrated clinical and proteomic-based model for diagnostic and prognostic prediction in pRCC.
PMID: 40437605
J Hematol Oncol · 2025
0.70
5
AKR1B10 Is a New Sensitive and Specific Marker for Fumarate Hydratase-Deficient Renal Cell Carcinoma.
PMID: 37580017
Mod Pathol · 2023
0.60