174 Genes
| Gene | Ensembl | Entrez | Aliases | Map Position | SwissProt ID | SwissProt Description | Subcellular Locations | Subcell PMIDs | Protein Complex | OMIM Gene | OMIM Disease |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ACADVL | ENSG00000072778 | 37 | ACAD6; LCACD; VLCAD | 17p13-p11 | P49748 | Acyl-CoA dehydrogenase, very-long-chain specific, mitochondrial precursor | Mitochondria Mitochondrial-inner-membrane | 7668252 | 609575 | VLCAD deficiency [201475] | |
| ACAT1 | ENSG00000075239 | 38 | ACAT; MAT; T2; THIL | 11q22.3-q23.1 | P24752 | Acetyl-CoA acetyltransferase, mitochondrial precursor | Mitochondria | 1979337 | 607809 | Alpha methylacetoacetic aciduria (MAT) [203750] | |
| ACSL4 | ENSG00000068366 | 2182 | ACS4; FACL4; LACS4; MRX63; MRX68 | Xq22.3-q23 | O60488 | Long-chain-fatty-acid-CoA ligase 4 | Microsome Mitochondria Mitochondrial-outer-membrane Peroxisome | 11889465 | 300157 | Mental retardation X linked (MRX63) [300387] | |
| ACSL6 | ENSG00000164398 | 23305 | ACS2; FACL6; FLJ16173; KIAA0837; LACS2; LACS5 | 5q31 | Q9UKU0 | Long-chain-fatty-acid-CoA ligase 6 | Microsome Mitochondria Mitochondrial-outer-membrane Peroxisome | 10548543 | 604443 | Acute myelogenous leukemia (AML) with eosinophilia[]; Acute eosinophilic leukemia (AEL) [604443] | |
| AGXT | ENSG00000172482 | 189 | AGT; AGT1; AGXT1; PH1; SPAT; SPT; TLH6 | 2q36-q37 | P21549 | Serine-pyruvate aminotransferase | Mitochondria Mitochondrial-matrix Peroxisome | 1703535 | 604285 | Primary hyperoxaluria type I (PH I) [259900] | |
| ALAS2 | ENSG00000158578 | 212 | ANH1; ASB; XLSA | Xp11.21 | P22557 | 5-aminolevulinic acid synthase, erythroid-specific, mitochondrial precursor | Mitochondria Mitochondrial-matrix | 10727444 | 301300 | X linked sideroblastic anemia (XLSA) [301300] | |
| ALDH18A1 | ENSG00000059573 | 5832 | GSAS; MGC117316; P5CS; PYCS | 10q24.3 | P54886 | Delta 1-pyrroline-5-carboxylate synthetase (P5CS) | Mitochondria Mitochondrial-inner-membrane | 10037775 15517380 | 138250 | Delta 1 pyrroline 5 carboxylate synthetase deficiency (P5CS) [138250] | |
| ALDH2 | ENSG00000111275 | 217 | ALDH-E2; ALDHI; ALDM; MGC1806 | 12q24.2 | P05091 | Aldehyde dehydrogenase, mitochondrial precursor | Mitochondria Mitochondrial-matrix | 7910607 | 100650 | Acute alcohol intolerance (AAI) [100650]; Uterine leiomyoma (UL) [150699]; Involutional osteoporosis [166710] |
