MGP Database

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MGP001461

Ontology/Pathway Information

Entrez Gene ID3141
Gene Nameholocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Gene Symbol HLCS
SpeciesHomo sapiens
Gene Ontology (GO Annotations)
GO IDSourceTypeDescription
GO:0000785 IDA:UniProtKBCchromatin
GO:0005737 IDA:HPACcytoplasm
GO:0005829 IDA:UniProtKBCcytosol
GO:0005739 IEA:UniProtKB-SubCellCmitochondrion
GO:0005652 IDA:UniProtKBCnuclear lamina
GO:0016363 IDA:UniProtKBCnuclear matrix
GO:0005524 IEA:UniProtKB-KWFATP binding
GO:0004077 IEA:UniProtKB-ECFbiotin-[acetyl-CoA-carboxylase] ligase activity
GO:0009374 IDA:UniProtKBFbiotin binding
GO:0004078 IEA:UniProtKB-ECFbiotin-[methylcrotonoyl-CoA-carboxylase] ligase activity
GO:0004079 IEA:UniProtKB-ECFbiotin-[methylmalonyl-CoA-carboxytransferase] ligase activity
GO:0004080 IDA:UniProtKBFbiotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase activity
GO:0018271 IDA:UniProtKBFbiotin-protein ligase activity
GO:0019899 IPI:UniProtKBFenzyme binding
GO:0006768 TAS:ReactomePbiotin metabolic process
GO:0008283 IMP:UniProtKBPcell proliferation
GO:0071110 IDA:UniProtKBPhistone biotinylation
GO:0016570 IDA:UniProtKBPhistone modification
GO:0009305 IDA:UniProtKBPprotein biotinylation
GO:0070781 IDA:UniProtKBPresponse to biotin
GO:0044281 TAS:ReactomePsmall molecule metabolic process
GO:0006766 TAS:ReactomePvitamin metabolic process
GO:0006767 TAS:ReactomePwater-soluble vitamin metabolic process
REACTOME Pathway Links
REACTOME Pathway IDDescription
REACT_11153Biotin transport and metabolism
REACT_169280Defective AMN causes hereditary megaloblastic anemia 1
REACT_169403Defective BTD causes biotidinase deficiency
REACT_169178Defective CD320 causes methylmalonic aciduria
REACT_169132Defective CUBN causes hereditary megaloblastic anemia 1
REACT_169415Defective GIF causes intrinsic factor deficiency
REACT_169312Defective HLCS causes multiple carboxylase deficiency
REACT_169363Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF
REACT_169316Defective MMAA causes methylmalonic aciduria type cblA
REACT_169318Defective MMAB causes methylmalonic aciduria type cblB
REACT_169169Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC
REACT_169256Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD
REACT_169149Defective MTR causes methylmalonic aciduria and homocystinuria type cblG
REACT_169439Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE
REACT_169313Defective MUT causes methylmalonic aciduria mut type
REACT_169120Defective TCN2 causes hereditary megaloblastic anemia
REACT_169238Defects in biotin (Btn) metabolism
REACT_169429Defects in cobalamin (B12) metabolism
REACT_169385Defects in vitamin and cofactor metabolism
REACT_116125Disease
REACT_111217Metabolism
REACT_11193Metabolism of vitamins and cofactors
REACT_11238Metabolism of water-soluble vitamins and cofactors
SMP Pathway Links
SMP IDDescription
SMP00174Biotinidase Deficiency
SMP00066Biotin Metabolism
SMP00564Multiple carboxylase deficiency, neonatal or early onset form
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