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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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ME2
malic enzyme 2
Chromosome 18 Β· 18q21.2
NCBI Gene: 4200Ensembl: ENSG00000082212.14HGNC: HGNC:6984UniProt: P23368
87PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
mitochondrionmalic enzyme activityregulation of NADP metabolic processmalate dehydrogenase (decarboxylating) (NADP+) activitydiabetes mellitusmajor depressive disorderliver diseaseaneurysm
✦AI Summary

ME2 (malic enzyme 2) is a NAD-dependent mitochondrial enzyme that catalyzes the oxidative decarboxylation of malate to pyruvate, playing a central role in metabolic regulation and mitochondrial function 1. ME2 exists in multiple cellular compartments with distinct functions: mitochondrial ME2 regulates the tricarboxylic acid (TCA) cycle and supports oxidative respiration 2, while cytoplasmic ME2 can function as a metabolic scaffold for glycolytic enzymes 2. ME2 activity is regulated through post-translational modifications, including desuccinylation by SIRT5 1, acetylation by ACAT1 3, and methylation by PRMT1 4. Beyond energy metabolism, fumarate binding to ME2 promotes mitochondrial biogenesis by facilitating dimer formation that activates nucleotide synthesis and mitoribosome assembly 5. In immune cells, ME2 is essential for CD8+ T cell activation and anti-tumor immunity through TCA cycle regulation 6. Clinically, ME2 dysregulation is implicated in multiple cancers (colorectal, ovarian, hepatocellular) where upregulation promotes mitochondrial respiration and chemoresistance 134, and genetic variants in ME2 are associated with genetic generalized epilepsy susceptibility 7.

Sources cited
1
ME2 catalyzes malate to pyruvate conversion; SIRT5-mediated desuccinylation at K346 activates ME2 to enhance mitochondrial respiration and promote colorectal cancer progression
PMID: 38007551
2
ME2 is critical for CD8+ T cell activation and anti-tumor immunity through TCA cycle regulation; ME2 depletion increases fumarate, which inhibits DAPK1
PMID: 39151423
3
AKT1 phosphorylates cytoplasmic ME2 at serine 9 to prevent mitochondrial translocation; cytoplasmic ME2 functions as a scaffold for glycolytic enzymes to promote glycolysis
PMID: 38263319
4
ACAT1-mediated acetylation of ME2 at K156 enhances its activity to produce lactate from glutamine, promoting chemoresistance in ovarian cancer
PMID: 39951294
5
Fumarate binding to ME2 promotes dimer formation, activating DUT for mtDNA synthesis and releasing mitoribosomal protein L45 for mitochondrial biogenesis
PMID: 33770508
6
ME2 variants are associated with adolescent-onset genetic generalized epilepsy susceptibility, with evidence linking ME2 expression to epilepsy risk
PMID: 30719716
7
PRMT1-mediated methylation of ME2 at K67 activates ME2 enzymatic activity, enhancing mitochondrial respiration and hepatocellular carcinoma growth
PMID: 39528487
Disease Associationsβ“˜20
diabetes mellitusOpen Targets
0.28Weak
liver diseaseOpen Targets
0.28Weak
major depressive disorderOpen Targets
0.28Weak
aneurysmOpen Targets
0.15Weak
aortic aneurysmOpen Targets
0.14Weak
hepatocellular carcinomaOpen Targets
0.10Suggestive
neoplasmOpen Targets
0.09Suggestive
glioblastoma multiformeOpen Targets
0.09Suggestive
breast cancerOpen Targets
0.08Suggestive
acute myeloid leukemiaOpen Targets
0.08Suggestive
gliomaOpen Targets
0.07Suggestive
oral squamous cell carcinomaOpen Targets
0.07Suggestive
hypertensionOpen Targets
0.04Suggestive
hyperpituitarismOpen Targets
0.03Suggestive
Increased blood pressureOpen Targets
0.03Suggestive
essential hypertensionOpen Targets
0.03Suggestive
cancerOpen Targets
0.03Suggestive
sign or symptomOpen Targets
0.03Suggestive
cardiovascular diseaseOpen Targets
0.02Suggestive
lung cancerOpen Targets
0.02Suggestive
Pathogenic Variants1
NM_002396.5(ME2):c.1379_1380del (p.Phe460fs)Likely pathogenic
See cases
β˜…β˜†β˜†β˜†β†’ Residue 460
View on ClinVar β†—
Related Genes
PKLRProtein interaction99%PKMProtein interaction99%PCProtein interaction97%ME3Protein interaction96%CSProtein interaction95%LDHCProtein interaction95%
Tissue Expression6 tissues
Heart
100%
Brain
59%
Bone Marrow
34%
Lung
26%
Ovary
19%
Liver
17%
Gene Interaction Network
Click a node to explore
ME2PKLRPKMPCME3CSLDHC
PROTEIN STRUCTURE
Preparing viewer…
PDB9AYI Β· 1.89 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.06LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.71 [0.48–1.06]
RankingsWhere ME2 stands among ~20K protein-coding genes
  • #5,494of 20,598
    Most Researched87
  • #4,935of 5,498
    Most Pathogenic Variants1
  • #10,733of 17,882
    Most Constrained (LOEUF)1.06
Genes detectedME2
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
SIRT5-mediated ME2 desuccinylation promotes cancer growth by enhancing mitochondrial respiration.
PMID: 38007551
Cell Death Differ Β· 2024
1.00
2
Malic enzyme 2 maintains metabolic state and anti-tumor immunity of CD8
PMID: 39151423
Mol Cell Β· 2024
0.90
3
AKT1 phosphorylation of cytoplasmic ME2 induces a metabolic switch to glycolysis for tumorigenesis.
PMID: 38263319
Nat Commun Β· 2024
0.80
4
ACAT1-Mediated ME2 Acetylation Drives Chemoresistance in Ovarian Cancer by Linking Glutaminolysis to Lactate Production.
PMID: 39951294
Adv Sci (Weinh) Β· 2025
0.70
5
Malic enzyme 2 connects the Krebs cycle intermediate fumarate to mitochondrial biogenesis.
PMID: 33770508
Cell Metab Β· 2021
0.60