Intracellular organelle lumen

Intracellular organelle lumen

• AASS: aminoadipate-semialdehyde synthase
• ACAD8: acyl-CoA dehydrogenase family, member 8
• ACADM: acyl-CoA dehydrogenase, C-4 to C-12 straight chain
• ACADS: acyl-CoA dehydrogenase, C-2 to C-3 short chain
• ACADSB: acyl-CoA dehydrogenase, short/branched chain
• ACADVL: acyl-CoA dehydrogenase, very long chain
• ACAN: aggrecan
• ACAT1: acetyl-CoA acetyltransferase 1
• ACTB: actin, beta
• AGPS: alkylglycerone phosphate synthase
• AGXT: alanine-glyoxylate aminotransferase
• AKT1: v-akt murine thymoma viral oncogene homolog 1
• ALAS1: aminolevulinate, delta-, synthase 1
• ALAS2: aminolevulinate, delta-, synthase 2
• ALDH4A1: aldehyde dehydrogenase 4 family, member A1
• ALDH5A1: aldehyde dehydrogenase 5 family, member A1
• ALDH7A1: aldehyde dehydrogenase 7 family, member A1
• ANG: angiogenin, ribonuclease, RNase A family, 5
• APOA1: apolipoprotein A-I
• APOB: apolipoprotein B
• APTX: aprataxin
• AR: androgen receptor
• ARID1A: AT rich interactive domain 1A (SWI-like)
• ARSA: arylsulfatase A
• ARSB: arylsulfatase B
• ARSE: arylsulfatase E (chondrodysplasia punctata 1)
• ASAH1: N-acylsphingosine amidohydrolase (acid ceramidase) 1
• ATM: ataxia telangiectasia mutated
• ATN1: atrophin 1
• ATRX: alpha thalassemia/mental retardation syndrome X-linked
• ATXN1: ataxin 1
• ATXN3: ataxin 3
• AUH: AU RNA binding protein/enoyl-CoA hydratase
• BCHE: butyrylcholinesterase
• 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
• C10orf2: chromosome 10 open reading frame 2
• CASQ2: calsequestrin 2 (cardiac muscle)
• CBS: cystathionine-beta-synthase
• CDC73: cell division cycle 73
• CHEK2: checkpoint kinase 2
• CIRH1A: cirrhosis, autosomal recessive 1A (cirhin)
• COL11A1: collagen, type XI, alpha 1
• COL11A2: collagen, type XI, alpha 2
• COL17A1: collagen, type XVII, alpha 1
• COL18A1: collagen, type XVIII, alpha 1
• COL1A1: collagen, type I, alpha 1
• COL1A2: collagen, type I, alpha 2
• COL2A1: collagen, type II, alpha 1
• COL3A1: collagen, type III, alpha 1
• COL4A1: collagen, type IV, alpha 1
• COL4A3: collagen, type IV, alpha 3 (Goodpasture antigen)
• COL4A4: collagen, type IV, alpha 4
• COL4A5: collagen, type IV, alpha 5
• COL5A1: collagen, type V, alpha 1
• COL5A2: collagen, type V, alpha 2
• COL6A1: collagen, type VI, alpha 1
• COL6A2: collagen, type VI, alpha 2
• COL6A3: collagen, type VI, alpha 3
• COL7A1: collagen, type VII, alpha 1
• COL8A2: collagen, type VIII, alpha 2
• COL9A1: collagen, type IX, alpha 1
• COL9A2: collagen, type IX, alpha 2
• COL9A3: collagen, type IX, alpha 3
• CPS1: carbamoyl-phosphate synthase 1, mitochondrial
• CPT2: carnitine palmitoyltransferase 2
• CREBBP: CREB binding protein
• CRTAP: cartilage associated protein
• CSTB: cystatin B (stefin B)
• CTDP1: CTD (carboxy-terminal domain, RNA polymerase II, polypeptide A) phosphatase, subunit 1
• CTNNB1: catenin (cadherin-associated protein), beta 1, 88kDa
• CTSA: cathepsin A
• CYP27A1: cytochrome P450, family 27, subfamily A, polypeptide 1
• D2HGDH: D-2-hydroxyglutarate dehydrogenase
• DARS2: aspartyl-tRNA synthetase 2, mitochondrial
• DBT: dihydrolipoamide branched chain transacylase E2
• DCN: decorin
• DGUOK: deoxyguanosine kinase
• DKC1: dyskeratosis congenita 1, dyskerin
• DLAT: dihydrolipoamide S-acetyltransferase
• DLD: dihydrolipoamide dehydrogenase
• EP300: E1A binding protein p300
• EPAS1: endothelial PAS domain protein 1
• ERAP1: endoplasmic reticulum aminopeptidase 1
• ERCC2: excision repair cross-complementing rodent repair deficiency, complementation group 2
• ERCC3: excision repair cross-complementing rodent repair deficiency, complementation group 3
• ERCC6: excision repair cross-complementing rodent repair deficiency, complementation group 6
• ERCC8: excision repair cross-complementing rodent repair deficiency, complementation group 8
• ESCO2: establishment of sister chromatid cohesion N-acetyltransferase 2
• ETFA: electron-transfer-flavoprotein, alpha polypeptide
• ETFB: electron-transfer-flavoprotein, beta polypeptide
• ETFDH: electron-transferring-flavoprotein dehydrogenase
• ETHE1: ethylmalonic encephalopathy 1
• EZH2: enhancer of zeste homolog 2 (Drosophila)
• F2: coagulation factor II (thrombin)
• F9: coagulation factor IX
• FANCA: Fanconi anemia, complementation group A
• FANCC: Fanconi anemia, complementation group C
• FANCG: Fanconi anemia, complementation group G
• FECH: ferrochelatase
• FH: fumarate hydratase
• FMR1: fragile X mental retardation 1
• FOXC1: forkhead box C1
• FOXC2: forkhead box C2 (MFH-1, mesenchyme forkhead 1)
• FOXF1: forkhead box F1
• FOXG1: forkhead box G1
• FOXL2: forkhead box L2
• FOXP3: forkhead box P3
• FUS: fused in sarcoma
• FXN: frataxin
• GALC: galactosylceramidase
• GALNS: galactosamine (N-acetyl)-6-sulfate sulfatase
• GARS: glycyl-tRNA synthetase
• GATA1: GATA binding protein 1 (globin transcription factor 1)
• GBA: glucosidase, beta, acid
• GCDH: glutaryl-CoA dehydrogenase
• GHRHR: growth hormone releasing hormone receptor
• GLA: galactosidase, alpha
• GLB1: galactosidase, beta 1
• GLI3: GLI family zinc finger 3
• GM2A: GM2 ganglioside activator
• GNPAT: glyceronephosphate O-acyltransferase
• GNS: glucosamine (N-acetyl)-6-sulfatase
• GPC3: glypican 3
• GRHPR: glyoxylate reductase/hydroxypyruvate reductase
• GUSB: glucuronidase, beta
• HADH: hydroxyacyl-CoA dehydrogenase
• HADHA: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
• HADHB: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
• HAX1: HCLS1 associated protein X-1
• HEXA: hexosaminidase A (alpha polypeptide)
• HEXB: hexosaminidase B (beta polypeptide)
• HLCS: holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
• HMGCL: 3-hydroxymethyl-3-methylglutaryl-CoA lyase
• HSD17B10: hydroxysteroid (17-beta) dehydrogenase 10
• IDS: iduronate 2-sulfatase
• IDUA: iduronidase, alpha-L-
• IKBKAP: inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase complex-associated protein
• INS: insulin
• ISCU: iron-sulfur cluster scaffold homolog (E. coli)
• IVD: isovaleryl-CoA dehydrogenase
• JAK2: Janus kinase 2
• KANSL1: KAT8 regulatory NSL complex subunit 1
• KAT6B: K(lysine) acetyltransferase 6B
• KMT2D: lysine (K)-specific methyltransferase 2D
• LEPRE1: leucine proline-enriched proteoglycan (leprecan) 1
• LMNA: lamin A/C
• MAPT: microtubule-associated protein tau
• MCCC1: methylcrotonoyl-CoA carboxylase 1 (alpha)
• MCEE: methylmalonyl CoA epimerase
• MCM6: minichromosome maintenance complex component 6
• MED12: mediator complex subunit 12
• MEN1: multiple endocrine neoplasia I
• MLH1: mutL homolog 1, colon cancer, nonpolyposis type 2 (E. coli)
• MMAA: methylmalonic aciduria (cobalamin deficiency) cblA type
• MSH2: mutS homolog 2, colon cancer, nonpolyposis type 1 (E. coli)
• MSH6: mutS homolog 6 (E. coli)
• MTTP: microsomal triglyceride transfer protein
• MUC1: mucin 1, cell surface associated
• MUT: methylmalonyl CoA mutase
• MUTYH: mutY homolog (E. coli)
• MYH7: myosin, heavy chain 7, cardiac muscle, beta
• MYO6: myosin VI
• NAGLU: N-acetylglucosaminidase, alpha
• NAGS: N-acetylglutamate synthase
• NBN: nibrin
• NCF2: neutrophil cytosolic factor 2
• NEU1: sialidase 1 (lysosomal sialidase)
• NF2: neurofibromin 2 (merlin)
• NGF: nerve growth factor (beta polypeptide)
• NIPBL: Nipped-B homolog (Drosophila)
• NOTCH2: notch 2
• NOTCH3: notch 3
• NR0B1: nuclear receptor subfamily 0, group B, member 1
• NR3C2: nuclear receptor subfamily 3, group C, member 2
• NR4A2: nuclear receptor subfamily 4, group A, member 2
• NR5A1: nuclear receptor subfamily 5, group A, member 1
• OAT: ornithine aminotransferase
• OTC: ornithine carbamoyltransferase
• OXCT1: 3-oxoacid CoA transferase 1
• PABPN1: poly(A) binding protein, nuclear 1
• PALB2: partner and localizer of BRCA2
• PAX6: paired box 6
• PAX8: paired box 8
• PC: pyruvate carboxylase
• PCCA: propionyl CoA carboxylase, alpha polypeptide
• PCCB: propionyl CoA carboxylase, beta polypeptide
• PDGFB: platelet-derived growth factor beta polypeptide
• PDHA1: pyruvate dehydrogenase (lipoamide) alpha 1
• PDHB: pyruvate dehydrogenase (lipoamide) beta
• PDHX: pyruvate dehydrogenase complex, component X
• PDP1: pyruvate dehydrogenase phosphatase catalytic subunit 1
• PEX7: peroxisomal biogenesis factor 7
• PHOX2A: paired-like homeobox 2a
• PHOX2B: paired-like homeobox 2b
• PHYH: phytanoyl-CoA 2-hydroxylase
• PITX2: paired-like homeodomain 2
• PML: promyelocytic leukemia
• POLG: polymerase (DNA directed), gamma
• POLH: polymerase (DNA directed), eta
• POLR1C: polymerase (RNA) I polypeptide C, 30kDa
• POLR1D: polymerase (RNA) I polypeptide D, 16kDa
• POLR3A: polymerase (RNA) III (DNA directed) polypeptide A, 155kDa
• POLR3B: polymerase (RNA) III (DNA directed) polypeptide B
• PPP1R12A: protein phosphatase 1, regulatory subunit 12A
• PRKAG2: protein kinase, AMP-activated, gamma 2 non-catalytic subunit
• PRNP: prion protein
• PROC: protein C (inactivator of coagulation factors Va and VIIIa)
• PROS1: protein S (alpha)
• PSAP: prosaposin
• RAD50: RAD50 homolog (S. cerevisiae)
• RAD51: RAD51 homolog (S. cerevisiae)
• RARA: retinoic acid receptor, alpha
• RARS2: arginyl-tRNA synthetase 2, mitochondrial
• RB1: retinoblastoma 1
• RBM8A: RNA binding motif protein 8A
• RPL11: ribosomal protein L11
• RPS10: ribosomal protein S10
• RPS19: ribosomal protein S19
• RPS24: ribosomal protein S24
• RPS6KA3: ribosomal protein S6 kinase, 90kDa, polypeptide 3
• RPS7: ribosomal protein S7
• RUNX2: runt-related transcription factor 2
• SALL1: sal-like 1 (Drosophila)
• SBDS: Shwachman-Bodian-Diamond syndrome
• SDHAF2: succinate dehydrogenase complex assembly factor 2
• SETX: senataxin
• SGSH: N-sulfoglucosamine sulfohydrolase
• SIX1: SIX homeobox 1
• SKI: v-ski sarcoma viral oncogene homolog (avian)
• SMAD3: SMAD family member 3
• SMAD4: SMAD family member 4
• SMARCA4: SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily a, member 4
• SMARCB1: SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily b, member 1
• SMARCE1: SWI/SNF related, matrix associated, actin dependent regulator of chromatin, subfamily e, member 1
• SMC1A: structural maintenance of chromosomes 1A
• SMC3: structural maintenance of chromosomes 3
• SMN2: survival of motor neuron 2, centromeric
• SMPD1: sphingomyelin phosphodiesterase 1, acid lysosomal
• SNAI2: snail family zinc finger 2
• SNCAIP: synuclein, alpha interacting protein
• SOD1: superoxide dismutase 1, soluble
• SOX2: SRY (sex determining region Y)-box 2
• SP110: SP110 nuclear body protein
• SPG11: spastic paraplegia 11 (autosomal recessive)
• SQSTM1: sequestosome 1
• STAT3: signal transducer and activator of transcription 3 (acute-phase response factor)
• SUCLA2: succinate-CoA ligase, ADP-forming, beta subunit
• SUCLG1: succinate-CoA ligase, alpha subunit
• T: T, brachyury homolog (mouse)
• TAF1: TAF1 RNA polymerase II, TATA box binding protein (TBP)-associated factor, 250kDa
• TBP: TATA box binding protein
• TBX5: T-box 5
• TCF4: transcription factor 4
• TCOF1: Treacher Collins-Franceschetti syndrome 1
• TERT: telomerase reverse transcriptase
• TGFB1: transforming growth factor, beta 1
• TGIF1: TGFB-induced factor homeobox 1
• TINF2: TERF1 (TRF1)-interacting nuclear factor 2
• TOR1A: torsin family 1, member A (torsin A)
• TP53: tumor protein p53
• TP63: tumor protein p63
• TTN: titin
• VCAN: versican
• VHL: von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase
• VRK1: vaccinia related kinase 1
• WHSC1: Wolf-Hirschhorn syndrome candidate 1
• WRN: Werner syndrome, RecQ helicase-like
• WT1: Wilms tumor 1
• XPA: xeroderma pigmentosum, complementation group A
• XPC: xeroderma pigmentosum, complementation group C
• ZEB2: zinc finger E-box binding homeobox 2

Source: Gene Ontology Consortium.