Intracellular organelle lumen

Intracellular organelle lumen

• ACADVL: acyl-coenzyme A dehydrogenase, very long chain
• ACAT1: acetyl-Coenzyme A acetyltransferase 1
• ALDH4A1: aldehyde dehydrogenase 4 family, member A1
• ANG: angiogenin, ribonuclease, RNase A family, 5
• APOB: apolipoprotein B (including Ag(x) antigen)
• APTX: aprataxin
• ATM: ataxia telangiectasia mutated
• BCKDHA: branched chain keto acid dehydrogenase E1, alpha polypeptide
• BCKDHB: branched chain keto acid dehydrogenase E1, beta polypeptide
• BLM: Bloom syndrome, RecQ helicase-like
• BRCA1: breast cancer 1, early onset
• BRCA2: breast cancer 2, early onset
• CBS: cystathionine-beta-synthase
• CHEK2: CHK2 checkpoint homolog (S. pombe)
• CPS1: carbamoyl-phosphate synthetase 1, mitochondrial
• CYP27A1: cytochrome P450, family 27, subfamily A, polypeptide 1
• DBT: dihydrolipoamide branched chain transacylase E2
• DLD: dihydrolipoamide dehydrogenase
• ERAP1: endoplasmic reticulum aminopeptidase 1
• ERCC6: excision repair cross-complementing rodent repair deficiency, complementation group 6
• ERCC8: excision repair cross-complementing rodent repair deficiency, complementation group 8
• ETFA: electron-transfer-flavoprotein, alpha polypeptide
• ETFB: electron-transfer-flavoprotein, beta polypeptide
• FECH: ferrochelatase (protoporphyria)
• FH: fumarate hydratase
• FMR1: fragile X mental retardation 1
• FXN: frataxin
• GLI3: GLI family zinc finger 3
• HADH: hydroxyacyl-Coenzyme A dehydrogenase
• HADHA: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit
• HADHB: hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit
• HMGCL: 3-hydroxymethyl-3-methylglutaryl-Coenzyme A lyase
• IKBKAP: inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase complex-associated protein
• IVD: isovaleryl Coenzyme A dehydrogenase
• MCCC1: methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
• MED12: mediator complex subunit 12
• MEN1: multiple endocrine neoplasia I
• MUTYH: mutY homolog (E. coli)
• MYH7: myosin, heavy chain 7, cardiac muscle, beta
• MYO6: myosin VI
• NAGS: N-acetylglutamate synthase
• NBN: nibrin
• NF2: neurofibromin 2 (merlin)
• OAT: ornithine aminotransferase (gyrate atrophy)
• OTC: ornithine carbamoyltransferase
• PABPN1: poly(A) binding protein, nuclear 1
• PAX8: paired box 8
• PC: pyruvate carboxylase
• PRKAG2: protein kinase, AMP-activated, gamma 2 non-catalytic subunit
• PROC: protein C (inactivator of coagulation factors Va and VIIIa)
• RAD51: RAD51 homolog (RecA homolog, E. coli) (S. cerevisiae)
• RB1: retinoblastoma 1
• RPL11: ribosomal protein L11
• RPS19: ribosomal protein S19
• RPS6KA3: ribosomal protein S6 kinase, 90kDa, polypeptide 3
• RPS7: ribosomal protein S7
• SBDS: Shwachman-Bodian-Diamond syndrome
• SERPINA1: serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1
• SETX: senataxin
• SIX1: SIX homeobox 1
• SMAD4: SMAD family member 4
• SMC3: structural maintenance of chromosomes 3
• SMN2: survival of motor neuron 2, centromeric
• SNCAIP: synuclein, alpha interacting protein
• SOD1: superoxide dismutase 1, soluble
• SUCLG1: succinate-CoA ligase, alpha subunit
• TAF1: TAF1 RNA polymerase II, TATA box binding protein (TBP)-associated factor, 250kDa
• TBP: TATA box binding protein
• TCOF1: Treacher Collins-Franceschetti syndrome 1
• TGFB1: transforming growth factor, beta 1
• TP53: tumor protein p53
• VWF: von Willebrand factor
• WRN: Werner syndrome, RecQ helicase-like

Source: Gene Ontology Consortium (June 2009).