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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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RCE1
Ras converting CAAX endopeptidase 1
Chromosome 11 · 11q13.2
NCBI Gene: 9986Ensembl: ENSG00000173653.8HGNC: HGNC:13721UniProt: Q0P5W4
29PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
endopeptidase activityexopeptidase activityprotein prenylationCAAX-box protein processingcolorectal carcinomaskeletal system diseaseneoplasmbreast cancer
✦AI Summary

RCE1 (Ras converting CAAX endopeptidase 1) is an integral membrane endoprotease localized to the endoplasmic reticulum that catalyzes proteolytic removal of the C-terminal three amino acids from prenylated CaaX proteins 1. The enzyme recognizes prenylated substrates through a glutamate-activated water mechanism and requires specific identity of the a2 amino residue, preferring Ile, Leu, or Val 1. Key substrates include Ras family GTPases, heterotrimeric G-protein gamma subunits, nuclear lamins, and protein kinases 1. RCE1 exists as multiple isoforms, with both isoform 1 and the N-terminally truncated isoform 2 required for proper H-Ras plasma membrane localization; USP17 regulates isoform 2 abundance through deubiquitination 2. Clinically, RCE1 exhibits tissue-dependent roles in cancer. In hepatocellular carcinoma and colorectal carcinoma, reduced RCE1 expression promotes epithelial-mesenchymal transition via P38/TGF-β signaling, enhancing invasion and metastasis, and independently predicts poor prognosis 345. Conversely, prostate cancer shows elevated RCE1 correlating with poor outcomes 6. RCE1 inhibition by anti-HIV protease inhibitors (ritonavir, lopinavir) impairs Rab protein processing, causing Golgi stress and hepatotoxicity 7. These findings establish RCE1 as both a potential therapeutic target and prognostic biomarker across multiple malignancies.

Sources cited
1
RCE1 is an integral membrane endoprotease that cleaves three C-terminal amino acids from prenylated CaaX proteins using a glutamate-activated water mechanism; recognizes substrates based on a2 amino acid identity
PMID: 29424242
2
Multiple RCE1 isoforms exist; both isoform 1 and N-terminally truncated isoform 2 are required for H-Ras plasma membrane localization; USP17 regulates isoform 2 through deubiquitination
PMID: 24134311
3
In hepatocellular carcinoma, reduced RCE1 expression promotes EMT via TGF-β1/H-Ras signaling, enhancing invasion and metastasis; RCE1 is an independent prognostic indicator
PMID: 31506952
4
RCE1 deficiency in hepatocellular carcinoma promotes EMT via P38 pathway activation, enhancing invasion and metastasis; predicts poor patient outcomes
PMID: 33312363
5
In colorectal carcinoma, reduced RCE1 expression predicts poor prognosis; RCE1 influences tumor invasion through the p38 pathway
PMID: 28615075
6
In prostate cancer, elevated RCE1 expression correlates with tumor progression, Gleason score, T class, and distant metastasis; predicts poor prognosis
PMID: 26546252
7
Anti-HIV protease inhibitors ritonavir and lopinavir suppress RCE1, impairing Rab13/Rab18 processing and causing Golgi dysfunction, organelle stress, and hepatotoxicity
PMID: 32490327
Disease Associationsⓘ20
colorectal carcinomaOpen Targets
0.07Suggestive
skeletal system diseaseOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
breast cancerOpen Targets
0.04Suggestive
estrogen-receptor positive breast cancerOpen Targets
0.04Suggestive
hepatocellular carcinomaOpen Targets
0.04Suggestive
bronchial diseaseOpen Targets
0.03Suggestive
preeclampsiaOpen Targets
0.03Suggestive
goutOpen Targets
0.02Suggestive
renal cell carcinomaOpen Targets
0.02Suggestive
diabetes mellitusOpen Targets
0.02Suggestive
type 2 diabetes mellitusOpen Targets
0.02Suggestive
posterior cortical atrophyOpen Targets
0.01Suggestive
rectum cancerOpen Targets
0.01Suggestive
prostate cancerOpen Targets
0.01Suggestive
tongue cancerOpen Targets
0.01Suggestive
benign prostatic hyperplasiaOpen Targets
0.01Suggestive
renal carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
cardiomyopathyOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SBK2Shared pathway100%FNTAProtein interaction97%FNTBProtein interaction97%ZMPSTE24Protein interaction96%NRASProtein interaction94%USP17L2Protein interaction86%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
46%
Liver
42%
Lung
35%
Brain
16%
Heart
13%
Gene Interaction Network
Click a node to explore
RCE1SBK2FNTAFNTBZMPSTE24NRASUSP17L2
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q0P5W4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.71LoF Tolerant
pLIⓘ
0.05Tolerant
Observed/Expected LoF0.46 [0.31–0.71]
RankingsWhere RCE1 stands among ~20K protein-coding genes
  • #12,220of 20,598
    Most Researched29
  • #5,469of 17,882
    Most Constrained (LOEUF)0.71
Genes detectedRCE1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Rce1: mechanism and inhibition.
PMID: 29424242
Crit Rev Biochem Mol Biol · 2018
1.00
2
Ritonavir and Lopinavir Suppress RCE1 and CAAX Rab Proteins Sensitizing the Liver to Organelle Stress and Injury.
PMID: 32490327
Hepatol Commun · 2020
0.90
3
Rce1 suppresses invasion and metastasis of hepatocellular carcinoma via epithelial-mesenchymal transition induced by the TGF-β1/H-Ras signaling pathway.
PMID: 31506952
J Cell Physiol · 2020
0.80
4
Reduced RCE1 expression predicts poor prognosis of colorectal carcinoma.
PMID: 28615075
BMC Cancer · 2017
0.70
5
Overexpressed Rce1 is positively correlated with tumor progression and predicts poor prognosis in prostate cancer.
PMID: 26546252
Hum Pathol · 2016
0.60