Process View
29 genes annotated in human
DOID:700
An inherited metabolic disorder that involves mitochondrial metabolism dysfunction.
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 "mitochondrial metabolism disease"
Name | Description | Probability | Func Analog Organism | |
---|---|---|---|---|
NDUFS3 | NADH dehydrogenase (ubiquinone) Fe-S protein 3, 30kDa (NADH-coenzyme Q reductase) | 0.993 | ||
NDUFS2 | NADH dehydrogenase (ubiquinone) Fe-S protein 2, 49kDa (NADH-coenzyme Q reductase) | 0.989 | ||
NDUFV2 | NADH dehydrogenase (ubiquinone) flavoprotein 2, 24kDa | 0.984 | ||
NDUFS8 | NADH dehydrogenase (ubiquinone) Fe-S protein 8, 23kDa (NADH-coenzyme Q reductase) | 0.974 | ||
NDUFV1 | NADH dehydrogenase (ubiquinone) flavoprotein 1, 51kDa | 0.969 | ||
NDUFB9 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9, 22kDa | 0.969 | ||
NDUFS7 | NADH dehydrogenase (ubiquinone) Fe-S protein 7, 20kDa (NADH-coenzyme Q reductase) | 0.967 | ||
NDUFA2 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2, 8kDa | 0.936 | ||
NDUFB8 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 8, 19kDa | 0.931 | ||
SDHB | succinate dehydrogenase complex, subunit B, iron sulfur (Ip) | 0.905 | ||
NDUFA9 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9, 39kDa | 0.850 | ||
CYC1 | cytochrome c-1 | 0.740 | ||
NDUFB10 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10, 22kDa | 0.442 | ||
SAMM50 | sorting and assembly machinery component 50 homolog (S. cerevisiae) | 0.374 | ||
NDUFB7 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7, 18kDa | 0.349 | ||
ATP5B | ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide | 0.278 | ||
NDUFA8 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa | 0.244 | ||
NDUFA12 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12 | 0.230 | ||
NDUFS6 | NADH dehydrogenase (ubiquinone) Fe-S protein 6, 13kDa (NADH-coenzyme Q reductase) | 0.220 | ||
NDUFA7 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7, 14.5kDa | 0.193 | ||
SFXN4 | sideroflexin 4 | 0.164 | ||
SDHA | succinate dehydrogenase complex, subunit A, flavoprotein (Fp) | 0.151 | ||
METTL17 | methyltransferase like 17 | 0.149 | ||
EIF2B3 | eukaryotic translation initiation factor 2B, subunit 3 gamma, 58kDa | 0.129 | ||
AIFM1 | apoptosis-inducing factor, mitochondrion-associated, 1 | 0.123 | ||
NDUFS1 | NADH dehydrogenase (ubiquinone) Fe-S protein 1, 75kDa (NADH-coenzyme Q reductase) | 0.103 | ||
ICT1 | immature colon carcinoma transcript 1 | 0.089 | ||
COQ3 | coenzyme Q3 homolog, methyltransferase (S. cerevisiae) | 0.079 | ||
EXOSC10 | exosome component 10 | 0.056 | ||
SUCLG1 | succinate-CoA ligase, alpha subunit | 0.055 | ||
UQCRFS1 | ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 | 0.049 | ||
UQCRB | ubiquinol-cytochrome c reductase binding protein | 0.049 | ||
NDUFA10 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 10, 42kDa | 0.048 | ||
IVD | isovaleryl-CoA dehydrogenase | 0.048 | ||
PET112L | PET112-like (yeast) | 0.046 | ||
GOT1 | glutamic-oxaloacetic transaminase 1, soluble (aspartate aminotransferase 1) | 0.045 | ||
SLC25A3 | solute carrier family 25 (mitochondrial carrier; phosphate carrier), member 3 | 0.036 | ||
OXA1L | oxidase (cytochrome c) assembly 1-like | 0.035 | ||
NFS1 | NFS1 nitrogen fixation 1 homolog (S. cerevisiae) | 0.035 | ||
COQ5 | coenzyme Q5 homolog, methyltransferase (S. cerevisiae) | 0.033 | ||
NDUFS4 | NADH dehydrogenase (ubiquinone) Fe-S protein 4, 18kDa (NADH-coenzyme Q reductase) | 0.033 | ||
TIMM13 | translocase of inner mitochondrial membrane 13 homolog (yeast) | 0.032 | ||
NDUFB5 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5, 16kDa | 0.031 | ||
COX16 | COX16 cytochrome c oxidase assembly homolog (S. cerevisiae) | 0.029 | ||
MCCC1 | methylcrotonoyl-CoA carboxylase 1 (alpha) | 0.028 | ||
MRPL46 | mitochondrial ribosomal protein L46 | 0.027 | ||
CBR4 | carbonyl reductase 4 | 0.026 | ||
NDUFAF1 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 1 | 0.024 | ||
ACAD9 | acyl-CoA dehydrogenase family, member 9 | 0.024 | ||
PCCB | propionyl CoA carboxylase, beta polypeptide | 0.023 | ||
ATP5O | ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit | 0.022 | ||
NDUFB6 | NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6, 17kDa | 0.022 | ||
SURF1 | surfeit 1 | 0.022 | ||
ACO2 | aconitase 2, mitochondrial | 0.022 | ||
HDHD2 | haloacid dehalogenase-like hydrolase domain containing 2 | 0.022 | ||
ATP5H | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d | 0.022 | ||
MRPL35 | mitochondrial ribosomal protein L35 | 0.021 | ||
CBARA1 | calcium binding atopy-related autoantigen 1 | 0.020 | ||
C20orf7 | chromosome 20 open reading frame 7 | 0.020 | ||
TYMP | thymidine phosphorylase | 0.020 | ||
MRPS21 | mitochondrial ribosomal protein S21 | 0.019 | ||
MGST3 | microsomal glutathione S-transferase 3 | 0.019 | ||
MRPS22 | mitochondrial ribosomal protein S22 | 0.018 | ||
MRPS36 | mitochondrial ribosomal protein S36 | 0.018 | ||
SDR39U1 | short chain dehydrogenase/reductase family 39U, member 1 | 0.018 | ||
ATP5C1 | ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 | 0.018 | ||
C1orf31 | chromosome 1 open reading frame 31 | 0.017 | ||
COX15 | COX15 homolog, cytochrome c oxidase assembly protein (yeast) | 0.017 | ||
NDUFA6 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6, 14kDa | 0.017 | ||
MRPL45 | mitochondrial ribosomal protein L45 | 0.017 | ||
USP30 | ubiquitin specific peptidase 30 | 0.017 | ||
NPRL2 | nitrogen permease regulator-like 2 (S. cerevisiae) | 0.016 | ||
ATP5G1 | ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9) | 0.016 | ||
MRPS11 | mitochondrial ribosomal protein S11 | 0.016 | ||
PIN4 | protein (peptidylprolyl cis/trans isomerase) NIMA-interacting, 4 (parvulin) | 0.016 | ||
NDUFA1 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1, 7.5kDa | 0.015 | ||
MRPL16 | mitochondrial ribosomal protein L16 | 0.015 | ||
PTCD2 | pentatricopeptide repeat domain 2 | 0.015 | ||
OXSM | 3-oxoacyl-ACP synthase, mitochondrial | 0.015 | ||
TIMM9 | translocase of inner mitochondrial membrane 9 homolog (yeast) | 0.015 | ||
MRPL15 | mitochondrial ribosomal protein L15 | 0.015 | ||
TMEM126A | transmembrane protein 126A | 0.014 | ||
CCDC56 | coiled-coil domain containing 56 | 0.014 | ||
PDHA1 | pyruvate dehydrogenase (lipoamide) alpha 1 | 0.014 | ||
DPH5 | DPH5 homolog (S. cerevisiae) | 0.014 | ||
DHODH | dihydroorotate dehydrogenase | 0.014 | ||
UQCRC1 | ubiquinol-cytochrome c reductase core protein I | 0.014 | ||
C8orf59 | chromosome 8 open reading frame 59 | 0.014 | ||
MRPS14 | mitochondrial ribosomal protein S14 | 0.013 | ||
COQ9 | coenzyme Q9 homolog (S. cerevisiae) | 0.013 | ||
GNPAT | glyceronephosphate O-acyltransferase | 0.013 | ||
SLC5A6 | solute carrier family 5 (sodium-dependent vitamin transporter), member 6 | 0.013 | ||
ISG15 | ISG15 ubiquitin-like modifier | 0.013 | ||
TACO1 | translational activator of mitochondrially encoded cytochrome c oxidase I | 0.013 | ||
TSFM | Ts translation elongation factor, mitochondrial | 0.013 | ||
HIF1AN | hypoxia inducible factor 1, alpha subunit inhibitor | 0.012 | ||
DERA | deoxyribose-phosphate aldolase (putative) | 0.012 | ||
RABEPK | Rab9 effector protein with kelch motifs | 0.012 | ||
IDH3B | isocitrate dehydrogenase 3 (NAD+) beta | 0.012 | ||
AFG3L2 | AFG3 ATPase family gene 3-like 2 (S. cerevisiae) | 0.012 | ||
NDUFV3 | NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa | 0.012 | ||
FOXRED1 | FAD-dependent oxidoreductase domain containing 1 | 0.012 | ||
COQ6 | coenzyme Q6 homolog, monooxygenase (S. cerevisiae) | 0.012 | ||
IFT46 | intraflagellar transport 46 homolog (Chlamydomonas) | 0.012 | ||
CCDC51 | coiled-coil domain containing 51 | 0.011 | ||
KLHDC2 | kelch domain containing 2 | 0.011 | ||
ECI1 | enoyl-CoA delta isomerase 1 | 0.011 | ||
C12orf45 | chromosome 12 open reading frame 45 | 0.011 | ||
MRPL52 | mitochondrial ribosomal protein L52 | 0.011 | ||
LARS2 | leucyl-tRNA synthetase 2, mitochondrial | 0.011 | ||
TMEM186 | transmembrane protein 186 | 0.011 | ||
MRRF | mitochondrial ribosome recycling factor | 0.011 | ||
NDUFS5 | NADH dehydrogenase (ubiquinone) Fe-S protein 5, 15kDa (NADH-coenzyme Q reductase) | 0.011 | ||
PFKM | phosphofructokinase, muscle | 0.011 | ||
SLC25A38 | solute carrier family 25, member 38 | 0.010 | ||
FARS2 | phenylalanyl-tRNA synthetase 2, mitochondrial | 0.010 | ||
OXNAD1 | oxidoreductase NAD-binding domain containing 1 | 0.010 | ||
UQCC | ubiquinol-cytochrome c reductase complex chaperone | 0.010 | ||
POLR3B | polymerase (RNA) III (DNA directed) polypeptide B | 0.010 | ||
C3orf1 | chromosome 3 open reading frame 1 | 0.010 | ||
C3orf75 | chromosome 3 open reading frame 75 | 0.010 | ||
ZCRB1 | zinc finger CCHC-type and RNA binding motif 1 | 0.010 | ||
FAM192A | family with sequence similarity 192, member A | 0.010 | ||
KIF20B | kinesin family member 20B | 0.010 | ||
COQ2 | coenzyme Q2 homolog, prenyltransferase (yeast) | 0.009 | ||
COX10 | COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast) | 0.007 | ||
BCS1L | BCS1-like (S. cerevisiae) | 0.006 | ||
ATPAF2 | ATP synthase mitochondrial F1 complex assembly factor 2 | 0.006 | ||
FAM36A | family with sequence similarity 36, member A | 0.005 | ||
TIMM8A | translocase of inner mitochondrial membrane 8 homolog A (yeast) | 0.005 | ||
ADCK3 | aarF domain containing kinase 3 | 0.004 | ||
C14orf153 | chromosome 14 open reading frame 153 | 0.004 | ||
PDSS2 | prenyl (decaprenyl) diphosphate synthase, subunit 2 | 0.003 | ||
C8orf38 | chromosome 8 open reading frame 38 | 0.003 | ||
PDSS1 | prenyl (decaprenyl) diphosphate synthase, subunit 1 | 0.002 | ||
C2orf64 | chromosome 2 open reading frame 64 | 0.002 | ||
SCO2 | SCO cytochrome oxidase deficient homolog 2 (yeast) | 0.002 | ||
LOC100131801 | hypothetical protein LOC100131801 | 0.001 | ||
NDUFAF2 | NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, assembly factor 2 | 0.001 |