Showing entry for Mitochondrial Diseases


　 　 　 　 　 　 　 　 　 　 　 　 　 　 　 　
General Disease Information
BXGD IdBXGD010539
Disease NameMitochondrial Diseases
Disease CUI IdC0751651
MeSH Codes C18  
Disease Class NameNutritional and Metabolic Diseases
Semantic TypeDisease or Syndrome
Human Phenotype Ontology Id   
Human Phenotype Ontology Term
Disease Ontology Id DOID:0014667   DOID:630  
Disease Ontology Class Namedisease of metabolism; genetic disease
Disorder Network disorder-protein-compound-food associations
The  disease-related  target proteins
Proteins
Uniport Id BXGT Id Protein Name Gene Status Protein Class
Uniport Id BXGT Id Protein name Gene Status Protein class
O00142 BXGT003883 Thymidine kinase 2, mitochondrial 7084 reviewed Kinase
O00217 BXGT003898 NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial 4728 reviewed Enzyme
O00429 BXGT003919 Dynamin-1-like protein 10059 reviewed Enzyme modulator
O00555 BXGT003936 Voltage-dependent P/Q-type calcium channel subunit alpha-1A 773 reviewed Ion channel
O15239 BXGT004139 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 4694 reviewed
O43181 BXGT004576 NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial 4724 reviewed
O43676 BXGT004611 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 4709 reviewed
O43678 BXGT004613 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 4695 reviewed Enzyme
O43772 BXGT004623 Mitochondrial carnitine/acylcarnitine carrier protein 788 reviewed Transporter
O43920 BXGT004637 NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 4725 reviewed
O60260 BXGT004864 E3 ubiquitin-protein ligase parkin 5071 reviewed Enzyme
O60313 BXGT004871 Dynamin-like 120 kDa protein, mitochondrial 4976 reviewed Enzyme modulator
O60885 BXGT004906 Bromodomain-containing protein 4 23476 reviewed Epigenetic regulator
O60930 BXGT004910 Ribonuclease H1 246243 reviewed
O75112 BXGT005099 LIM domain-binding protein 3 11155 reviewed Cellular structure
O75251 BXGT005107 NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial 374291 reviewed
O75306 BXGT005108 NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial 4720 reviewed Enzyme
O75380 BXGT005120 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial 4726 reviewed Enzyme
O75390 BXGT005123 Citrate synthase, mitochondrial 1431 reviewed Enzyme
O75489 BXGT005131 NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial 4722 reviewed
O75880 BXGT005158 Protein SCO1 homolog, mitochondrial 6341 reviewed Enzyme
O95169 BXGT005356 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial 4714 reviewed Enzyme
O95299 BXGT005372 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial 4705 reviewed Enzyme
O95363 BXGT005375 Phenylalanine--tRNA ligase, mitochondrial 10667 reviewed Enzyme
O95831 BXGT005399 Apoptosis-inducing factor 1, mitochondrial 9131 reviewed Enzyme
P00156 BXGT005469 Cytochrome b 4519 reviewed
P00480 BXGT005551 Ornithine carbamoyltransferase, mitochondrial 5009 reviewed
P00846 BXGT005674 ATP synthase subunit a 4508 reviewed Transporter
P01009 BXGT005714 Alpha-1-antitrypsin 5265 reviewed Enzyme modulator
P01024 BXGT005719 Complement C3 718 reviewed Enzyme modulator
P01375 BXGT005753 Tumor necrosis factor 7124 reviewed Signaling
P02144 BXGT005853 Myoglobin 4151 reviewed
P02462 BXGT005881 Collagen alpha-1(IV) chain 1282 reviewed
P02766 BXGT005953 Transthyretin 7276 reviewed Transporter
P02768 BXGT005955 Serum albumin 213 reviewed Transporter
P03886 BXGT006108 NADH-ubiquinone oxidoreductase chain 1 4535 reviewed Enzyme
P03897 BXGT006111 NADH-ubiquinone oxidoreductase chain 3 4537 reviewed
P03905 BXGT006113 NADH-ubiquinone oxidoreductase chain 4 4538 reviewed Enzyme
P03915 BXGT006114 NADH-ubiquinone oxidoreductase chain 5 4540 reviewed Enzyme
P04179 BXGT006163 Superoxide dismutase [Mn], mitochondrial 6648 reviewed Enzyme
P04732 BXGT006252 Metallothionein-1E 4493 reviewed
P04792 BXGT006264 Heat shock protein beta-1 3315 reviewed
P05091 BXGT006312 Aldehyde dehydrogenase, mitochondrial 217 reviewed Enzyme
P07203 BXGT006566 Glutathione peroxidase 1 2876 reviewed Enzyme
P07741 BXGT006666 Adenine phosphoribosyltransferase 353 reviewed
P08559 BXGT006788 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial 5160 reviewed
P08922 BXGT006843 Proto-oncogene tyrosine-protein kinase ROS 6098 reviewed Kinase
P09601 BXGT006909 Heme oxygenase 1 3162 reviewed
P10646 BXGT007598 Tissue factor pathway inhibitor 7035 reviewed Enzyme modulator
P12235 BXGT007774 ADP/ATP translocase 1 291 reviewed Transporter
P12236 BXGT007775 ADP/ATP translocase 3 293 reviewed Transporter
P14207 BXGT007993 Folate receptor beta 2350 reviewed
P15289 BXGT008102 Arylsulfatase A 410 reviewed Enzyme
P15328 BXGT008108 Folate receptor alpha 2348 reviewed
P18440 BXGT008404 Arylamine N-acetyltransferase 1 9 reviewed Enzyme
P19440 BXGT008481 Glutathione hydrolase 1 proenzyme 2678 reviewed Enzyme
P19971 BXGT008537 Thymidine phosphorylase 1890 reviewed Enzyme
P20132 BXGT008546 L-serine dehydratase/L-threonine deaminase 10993 reviewed Enzyme
P21980 BXGT008715 Protein-glutamine gamma-glutamyltransferase 2 7052 reviewed Enzyme
P23443 BXGT008866 Ribosomal protein S6 kinase beta-1 6198 reviewed Kinase
P23786 BXGT008906 Carnitine O-palmitoyltransferase 2, mitochondrial 1376 reviewed Enzyme
P28331 BXGT009252 NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial 4719 reviewed Enzyme
P31040 BXGT009503 Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 6389 reviewed Enzyme
P33527 BXGT009728 Multidrug resistance-associated protein 1 4363 reviewed Transporter
P35228 BXGT009800 Nitric oxide synthase, inducible 4843 reviewed
P35498 BXGT009838 Sodium channel protein type 1 subunit alpha 6323 reviewed Ion channel
P35610 BXGT009855 Sterol O-acyltransferase 1 6646 reviewed Enzyme
P35914 BXGT009880 Hydroxymethylglutaryl-CoA lyase, mitochondrial 3155 reviewed Enzyme
P36551 BXGT009927 Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial 1371 reviewed Enzyme
P41250 BXGT010244 Glycine--tRNA ligase 2617 reviewed Enzyme
P42345 BXGT010304 Serine/threonine-protein kinase mTOR 2475 reviewed Kinase
P42574 BXGT010317 Caspase-3 836 reviewed Enzyme
P45381 BXGT010460 Aspartoacylase 443 reviewed
P49821 BXGT010748 NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial 4723 reviewed
P51970 BXGT010912 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 4702 reviewed Enzyme
P54098 BXGT011030 DNA polymerase subunit gamma-1 5428 reviewed Enzyme
P55157 BXGT011108 Microsomal triglyceride transfer protein large subunit 4547 reviewed Transporter
P55789 BXGT011126 FAD-linked sulfhydryl oxidase ALR 2671 reviewed Enzyme
P56181 BXGT011148 NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial 4731 reviewed
P56556 BXGT011179 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 4700 reviewed Enzyme
P60709 BXGT011322 Actin, cytoplasmic 1 60 reviewed Cellular structure
Q00059 BXGT012512 Transcription factor A, mitochondrial 7019 reviewed
Q00535 BXGT012534 Cyclin-dependent-like kinase 5 1020 reviewed Kinase
Q00796 BXGT012549 Sorbitol dehydrogenase 6652 reviewed Enzyme
Q01094 BXGT012568 Transcription factor E2F1 1869 reviewed Nucleic acid binding
Q04446 BXGT012764 1,4-alpha-glucan-branching enzyme 2632 reviewed Enzyme
Q05655 BXGT012832 Protein kinase C delta type 5580 reviewed Kinase
Q07021 BXGT012915 Complement component 1 Q subcomponent-binding protein, mitochondrial 708 reviewed
Q08211 BXGT012970 ATP-dependent RNA helicase A 1660 reviewed Enzyme
Q08257 BXGT012975 Quinone oxidoreductase 1429 reviewed
Q09013 BXGT013023 Myotonin-protein kinase 1760 reviewed Kinase
Q12778 BXGT013263 Forkhead box protein O1 2308 reviewed
Q12882 BXGT013276 Dihydropyrimidine dehydrogenase [NADP(+)] 1806 reviewed Enzyme
Q13085 BXGT013309 Acetyl-CoA carboxylase 1 31 reviewed
Q13164 BXGT013322 Mitogen-activated protein kinase 7 5598 reviewed Kinase
Q13285 BXGT013335 Steroidogenic factor 1 2516 reviewed Nuclear receptor
Q13472 BXGT013360 DNA topoisomerase 3-alpha 7156 reviewed Enzyme
Q13547 BXGT013373 Histone deacetylase 1 3065 reviewed Epigenetic regulator
Q14315 BXGT013460 Filamin-C 2318 reviewed
Q15046 BXGT013527 Lysine--tRNA ligase 3735 reviewed Enzyme
Q15067 BXGT013532 Peroxisomal acyl-coenzyme A oxidase 1 51 reviewed Enzyme
Q16236 BXGT013624 Nuclear factor erythroid 2-related factor 2 4780 reviewed Enzyme
Q16548 BXGT013636 Bcl-2-related protein A1 597 reviewed Signaling
Q16595 BXGT013643 Frataxin, mitochondrial 2395 reviewed Enzyme
Q16740 BXGT013664 ATP-dependent Clp protease proteolytic subunit, mitochondrial 8192 reviewed
Q16881 BXGT013688 Thioredoxin reductase 1, cytoplasmic 7296 reviewed Enzyme
Q7LG56 BXGT016999 Ribonucleoside-diphosphate reductase subunit M2 B 50484 reviewed Enzyme
Q7Z2E3 BXGT017246 Aprataxin 54840 reviewed Nucleic acid binding
Q86Y39 BXGT017632 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 126328 reviewed
Q8IWA4 BXGT018353 Mitofusin-1 55669 reviewed Enzyme
Q8IYB8 BXGT018365 ATP-dependent RNA helicase SUPV3L1, mitochondrial 6832 reviewed
Q8N183 BXGT018497 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2 91942 reviewed
Q8NI60 BXGT018572 Atypical kinase COQ8A, mitochondrial 56997 reviewed Kinase
Q8WVQ1 BXGT018996 Soluble calcium-activated nucleotidase 1 124583 reviewed
Q96EB6 BXGT019646 NAD-dependent protein deacetylase sirtuin-1 23411 reviewed Epigenetic regulator
Q99551 BXGT019933 Transcription termination factor 1, mitochondrial 7978 reviewed
Q99714 BXGT019954 3-hydroxyacyl-CoA dehydrogenase type-2 3028 reviewed Enzyme
Q9BQP7 BXGT020114 Mitochondrial genome maintenance exonuclease 1 92667 reviewed
Q9BU61 BXGT020131 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 3 25915 reviewed
Q9C000 BXGT020206 NACHT, LRR and PYD domains-containing protein 1 22861 reviewed
Q9NSE4 BXGT021207 Isoleucine--tRNA ligase, mitochondrial 55699 reviewed Enzyme
Q9NTG7 BXGT021211 NAD-dependent protein deacetylase sirtuin-3, mitochondrial 23410 reviewed Epigenetic regulator
Q9NX14 BXGT021242 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial 54539 reviewed Enzyme
Q9P032 BXGT021286 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4 29078 reviewed
Q9P0J0 BXGT021287 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 51079 reviewed Enzyme
Q9UGM6 BXGT021687 Tryptophan--tRNA ligase, mitochondrial 10352 reviewed
Q9UM47 BXGT021772 Neurogenic locus notch homolog protein 3 4854 reviewed
Q9Y2Z4 BXGT022251 Tyrosine--tRNA ligase, mitochondrial 51067 reviewed Enzyme
Q9Y375 BXGT022253 Complex I intermediate-associated protein 30, mitochondrial 51103 reviewed
Q9Y606 BXGT022312 tRNA pseudouridine synthase A 80324 reviewed Enzyme
O60936 BXGT022857 Nucleolar protein 3 8996 reviewed
Q7L0Y3 BXGT023259 tRNA methyltransferase 10 homolog C 54931 reviewed
O75208 BXGT023882 Ubiquinone biosynthesis protein COQ9, mitochondrial 57017 reviewed
O95140 BXGT024482 Mitofusin-2 9927 reviewed Enzyme
P38646 BXGT024954 Stress-70 protein, mitochondrial 3313 reviewed
Q9Y4W6 BXGT025025 AFG3-like protein 2 10939 reviewed Enzyme
Q9H1K1 BXGT025766 Iron-sulfur cluster assembly enzyme ISCU, mitochondrial 23479 reviewed
Q8N122 BXGT026091 Regulatory-associated protein of mTOR 57521 reviewed
P00403 BXGT026137 Cytochrome c oxidase subunit 2 4513 reviewed Enzyme
The  disease-related  compounds
Compounds
BXGC Id Compound Name Structure Molecular Weight Number of targets?Number of times compound connecting the disease , higher numbers indicate the compound is more associated with the disease
The  disease-related  foods
Foods
BXGF Id Food Name Scientific Name Food Group Number of compounds in food?Number of compounds in food that connecting disease, higher numbers indicate the more compounds in food are more associated with the dusease