BTD

Biotinidase functions to recycle biotin in the body by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. Biotinidase has also been shown to have biotinyl-transferase activity. Defects in the biotinidase gene cause multiple carboxylase deficiency. [provided by RefSeq]
Full Name
biotinidase
Function
Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
Biological Process
Biotin metabolic process Source: GO_Central
Central nervous system development Source: ProtInc
Cellular Location
Extracellular space
Involvement in disease
Biotinidase deficiency (BTD deficiency): A juvenile form of multiple carboxylase deficiency, an autosomal recessive disorder of biotin metabolism, characterized by ketoacidosis, hyperammonemia, excretion of abnormal organic acid metabolites, and dermatitis. Biotinidase deficiency is characterized by seizures, hypotonia, skin rash, alopecia, ataxia, hearing loss, and optic atrophy. If untreated, symptoms usually become progressively worse, and coma and death may occur.
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Anti-BTD antibodies

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Mouse Anti-BTD Recombinant Antibody (3B10-2B3) (CBMAB-A0897-LY)

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Target: BTD
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 3B10-2B3
Application*: WB, E
Datasheet SDS

Mouse Anti-BTD Recombinant Antibody (CBYY-2026) (CBMAB-2375-YY)

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Target: BTD
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBYY-2026
Application*: E
Datasheet SDS

Human Recombinant BTD protein, His Tag (V2LY-0526-LY2419)

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Target: BTD
Expressed Host: HEK293 Cells
Sequence: Amino Acid: 1-543
Tag: His Tag
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IGImmunochromatography
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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