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MitoProteome Human Mitochondrial Protein Database
MitoProteome Database
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Ontology/Pathway Information
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Domain Information
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UniProt Annotations
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Related Proteins
MT000093
Gene Information
Entrez Gene ID
686
Gene Name
biotinidase
Gene Symbol
BTD
Species
Homo sapiens
Gene Ontology (GO Annotations)
GO ID
Source
Type
Description
GO:0045177
IEA:Ensembl
C
apical part of cell
GO:0005576
TAS:Reactome
C
extracellular region
GO:0005615
IDA:UniProt
C
extracellular space
GO:0070062
IDA:UniProtKB
C
extracellular vesicular exosome
GO:0005759
TAS:Reactome
C
mitochondrial matrix
GO:0005730
IEA:Ensembl
C
nucleolus
GO:0043204
IEA:Ensembl
C
perikaryon
GO:0004075
TAS:ProtInc
F
biotin carboxylase activity
GO:0047708
IEA:UniProtKB-EC
F
biotinidase activity
GO:0006768
TAS:Reactome
P
biotin metabolic process
GO:0007417
TAS:ProtInc
P
central nervous system development
GO:0008544
TAS:ProtInc
P
epidermis development
GO:0044281
TAS:Reactome
P
small molecule metabolic process
GO:0006766
TAS:Reactome
P
vitamin metabolic process
GO:0006767
TAS:Reactome
P
water-soluble vitamin metabolic process
KEGG Pathway Links
KEGG Pathway ID
Description
hsa00780
Biotin metabolism
ko00780
Biotin metabolism
hsa01100
Metabolic pathways
hsa04977
Vitamin digestion and absorption
ko04977
Vitamin digestion and absorption
REACTOME Pathway Links
REACTOME Pathway ID
Description
REACT_11153
Biotin transport and metabolism
REACT_169280
Defective AMN causes hereditary megaloblastic anemia 1
REACT_169403
Defective BTD causes biotidinase deficiency
REACT_169178
Defective CD320 causes methylmalonic aciduria
REACT_169132
Defective CUBN causes hereditary megaloblastic anemia 1
REACT_169415
Defective GIF causes intrinsic factor deficiency
REACT_169312
Defective HLCS causes multiple carboxylase deficiency
REACT_169363
Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF
REACT_169316
Defective MMAA causes methylmalonic aciduria type cblA
REACT_169318
Defective MMAB causes methylmalonic aciduria type cblB
REACT_169169
Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC
REACT_169256
Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD
REACT_169149
Defective MTR causes methylmalonic aciduria and homocystinuria type cblG
REACT_169439
Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE
REACT_169313
Defective MUT causes methylmalonic aciduria mut type
REACT_169120
Defective TCN2 causes hereditary megaloblastic anemia
REACT_169238
Defects in biotin (Btn) metabolism
REACT_169429
Defects in cobalamin (B12) metabolism
REACT_169385
Defects in vitamin and cofactor metabolism
REACT_116125
Disease
REACT_111217
Metabolism
REACT_11193
Metabolism of vitamins and cofactors
REACT_11238
Metabolism of water-soluble vitamins and cofactors
MitoProteome Human Mitochondrial Protein Database
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