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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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GRPEL2
GrpE like 2, mitochondrial
Chromosome 5 · 5q32
NCBI Gene: 134266Ensembl: ENSG00000164284.16HGNC: HGNC:21060UniProt: Q8TAA5
28PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingmitochondrionadenyl-nucleotide exchange factor activityPAM complex, Tim23 associated import motorgliomacolorectal carcinomaneoplasmdilated cardiomyopathy
✦AI Summary

GRPEL2 is a mitochondrial nucleotide exchange factor essential for regulating mitochondrial protein import and quality control. As a component of the PAM complex, GRPEL2 facilitates the translocation of transit peptide-containing proteins into the mitochondrial matrix by modulating mtHSP70's nucleotide-binding cycle 1. GRPEL2 associates with mtHSP70 as a hetero-oligomeric subcomplex, helping fine-tune chaperone activity and regulate crucial functions including preprotein import and Fe-S cluster biogenesis 1. However, GRPEL2 exhibits preferential binding to ADP-bound mtHSP70 compared to GRPEL1 and lacks the capacity to enhance mtHSP70 ATPase activity 2. GRPEL2 functions as a stress-regulated redox sensor, with its Cys87 residue serving as a thiol switch that promotes dimerization under oxidative stress conditions 3. This redox regulation appears to enhance mitochondrial proteostasis during cellular stress 3. Clinically, GRPEL2 is upregulated in multiple cancers including glioblastoma, hepatocellular carcinoma, and esophageal squamous cell carcinoma, correlating with poor prognosis 4567. In glioblastoma, GRPEL2 knockdown inhibits proliferation and induces cell cycle arrest through reduced oxidative respiration 4. In esophageal cancer, GRPEL2 depletion triggers apoptosis via JNK pathway activation 7. Additionally, GRPEL2 hypomethylation has been associated with vinyl chloride-induced DNA damage 8.

Sources cited
1
GRPEL2 is a nucleotide exchange factor that regulates mtHSP70 function and controls mitochondrial protein import and Fe-S cluster biogenesis through hetero-oligomeric association with mtHSP70
PMID: 28848044
2
GRPEL2 shows lower affinity for ADP-bound mtHSP70 compared to GRPEL1 and lacks capacity to enhance mtHSP70 ATPase activity
PMID: 39445986
3
GRPEL2 is redox-regulated with Cys87 as a thiol switch that promotes intermolecular disulfide bonding and dimerization under oxidative stress
PMID: 30098457
4
GRPEL2 is upregulated in glioblastoma correlating with poor prognosis; GRPEL2 knockdown inhibits proliferation and reduces oxidative respiration
PMID: 34884508
5
GRPEL2 is upregulated in liver cancer tissues and serves as a key gene for prognostic and diagnostic models
PMID: 40465781
6
GRPEL2 is included in a ten-gene UPR-related prognostic signature model for hepatocellular carcinoma
PMID: 37587817
7
GRPEL2 is upregulated in esophageal squamous cell carcinoma; GRPEL2 knockdown induces apoptosis via JNK/MAPK pathway activation
PMID: 40499524
8
GRPEL2 hypomethylation is associated with vinyl chloride-induced DNA damage
PMID: 35548910
Disease Associationsⓘ20
colorectal carcinomaOpen Targets
0.07Suggestive
gliomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.06Suggestive
dilated cardiomyopathyOpen Targets
0.06Suggestive
glioblastoma multiformeOpen Targets
0.04Suggestive
diabetes mellitusOpen Targets
0.04Suggestive
esophageal squamous cell carcinomaOpen Targets
0.03Suggestive
hepatocellular carcinomaOpen Targets
0.03Suggestive
breast benign neoplasmOpen Targets
0.03Suggestive
ankylosing spondylitisOpen Targets
0.03Suggestive
Subdural hemorrhageOpen Targets
0.03Suggestive
disease of peritoneumOpen Targets
0.02Suggestive
glioblastomaOpen Targets
0.02Suggestive
colorectal cancerOpen Targets
0.01Suggestive
diabetic cardiomyopathyOpen Targets
0.01Suggestive
liver cancerOpen Targets
0.01Suggestive
Myocardial IschemiaOpen Targets
0.01Suggestive
oligodendrogliomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
esophageal carcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HSPD1Protein interaction93%HSPE1Protein interaction93%LONP1Protein interaction93%HSPA4LProtein interaction93%CLPBProtein interaction93%DNAJA3Protein interaction83%
Tissue Expression6 tissues
Lung
100%
Brain
98%
Heart
85%
Bone Marrow
64%
Liver
55%
Ovary
46%
Gene Interaction Network
Click a node to explore
GRPEL2HSPD1HSPE1LONP1HSPA4LCLPBDNAJA3
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8TAA5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.23LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.80 [0.53–1.23]
RankingsWhere GRPEL2 stands among ~20K protein-coding genes
  • #12,360of 20,598
    Most Researched28
  • #12,911of 17,882
    Most Constrained (LOEUF)1.23
Genes detectedGRPEL2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Regulation of mitochondrial protein import by the nucleotide exchange factors GrpEL1 and GrpEL2 in human cells.
PMID: 28848044
J Biol Chem · 2017
1.00
2
Identification of key genes and signaling pathways of liver cancer and model construction for prognosis and diagnosis based on bioinformatics analysis.
PMID: 40465781
PLoS One · 2025
0.90
3
Preferential binding of ADP-bound mitochondrial HSP70 to the nucleotide exchange factor GRPEL1 over GRPEL2.
PMID: 39445986
Protein Sci · 2024
0.80
4
GRPEL2 Knockdown Exerts Redox Regulation in Glioblastoma.
PMID: 34884508
Int J Mol Sci · 2021
0.70
5
GRPEL2 Modulates Apoptosis in Esophageal Squamous Cell Carcinoma via the JNK Signaling Pathway.
PMID: 40499524
Mol Carcinog · 2025
0.60