IMP
Process View
Homo sapiens
14 genes annotated in human
organic acidemia
DOID:0060159
An amino acid metabolic disorder that disrupts normal amino acid metabolism causing a building up of branched-chain amino acids.
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In addition to gene-name show these genes:
Click on a gene's description to view its network relationships with genes known to be involved in "organic acidemia"
| Name | Description | Probability | Func Analog Organism | |
|---|---|---|---|---|
| UQCRC2 | ubiquinol-cytochrome c reductase core protein II | 0.743 | ||
| NDUFS1 | NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) | 0.683 | ||
| BCKDHB | branched chain keto acid dehydrogenase E1, beta polypeptide | 0.167 | ||
| PDHX | pyruvate dehydrogenase complex, component X | 0.156 | ||
| MCCC1 | methylcrotonoyl-CoA carboxylase 1 (alpha) | 0.123 | ||
| BCKDK | branched chain ketoacid dehydrogenase kinase | 0.120 | ||
| EIF2C4 | eukaryotic translation initiation factor 2C, 4 | 0.049 | ||
| DLAT | dihydrolipoamide S-acetyltransferase | 0.044 | ||
| BCKDHA | branched chain keto acid dehydrogenase E1, alpha polypeptide | 0.042 | ||
| SLC25A46 | solute carrier family 25, member 46 | 0.040 | ||
| MRPS30 | mitochondrial ribosomal protein S30 | 0.029 | ||
| ACADM | acyl-CoA dehydrogenase, C-4 to C-12 straight chain | 0.027 | ||
| ADI1 | acireductone dioxygenase 1 | 0.023 | ||
| PEX7 | peroxisomal biogenesis factor 7 | 0.022 | ||
| ETFDH | electron-transferring-flavoprotein dehydrogenase | 0.022 | ||
| TRAPPC6A | trafficking protein particle complex 6A | 0.021 | ||
| CPT2 | carnitine palmitoyltransferase 2 | 0.021 | ||
| MDH1 | malate dehydrogenase 1, NAD (soluble) | 0.020 | ||
| HIBADH | 3-hydroxyisobutyrate dehydrogenase | 0.020 | ||
| ECI1 | enoyl-CoA delta isomerase 1 | 0.019 | ||
| RSL24D1 | ribosomal L24 domain containing 1 | 0.017 | ||
| NNT | nicotinamide nucleotide transhydrogenase | 0.016 | ||
| ALDH6A1 | aldehyde dehydrogenase 6 family, member A1 | 0.016 | ||
| FDX1 | ferredoxin 1 | 0.015 | ||
| ACAT1 | acetyl-CoA acetyltransferase 1 | 0.014 | ||
| C21orf33 | chromosome 21 open reading frame 33 | 0.013 | ||
| PDCD10 | programmed cell death 10 | 0.013 | ||
| ACAD8 | acyl-CoA dehydrogenase family, member 8 | 0.012 | ||
| GHITM | growth hormone inducible transmembrane protein | 0.012 | ||
| SUCLA2 | succinate-CoA ligase, ADP-forming, beta subunit | 0.012 | ||
| CCNH | cyclin H | 0.012 | ||
| GCDH | glutaryl-CoA dehydrogenase | 0.012 | ||
| DLD | dihydrolipoamide dehydrogenase | 0.011 | ||
| HIBCH | 3-hydroxyisobutyryl-CoA hydrolase | 0.011 | ||
| HMGCL | 3-hydroxymethyl-3-methylglutaryl-CoA lyase | 0.011 | ||
| MIPEP | mitochondrial intermediate peptidase | 0.010 | ||
| PTS | 6-pyruvoyltetrahydropterin synthase | 0.010 | ||
| TMEM59 | transmembrane protein 59 | 0.010 | ||
| DBT | dihydrolipoamide branched chain transacylase E2 | 0.006 | ||
| IVD | isovaleryl-CoA dehydrogenase | 0.006 | ||
| PCCA | propionyl CoA carboxylase, alpha polypeptide | 0.005 | ||
| MMAB | methylmalonic aciduria (cobalamin deficiency) cblB type | 0.005 | ||
| MMAA | methylmalonic aciduria (cobalamin deficiency) cblA type | 0.004 | ||
| MMACHC | methylmalonic aciduria (cobalamin deficiency) cblC type, with homocystinuria | 0.003 | ||
| PCCB | propionyl CoA carboxylase, beta polypeptide | 0.003 | ||
| PPM1K | protein phosphatase, Mg2+/Mn2+ dependent, 1K | 0.002 | ||
| HGD | homogentisate 1,2-dioxygenase | 0.002 | ||
| MMADHC | methylmalonic aciduria (cobalamin deficiency) cblD type, with homocystinuria | 0.001 | ||
| LMBRD1 | LMBR1 domain containing 1 | 0.001 |