Product Name
Biotinidase (BTD), Polyclonal Antibody
Full Product Name
Anti-Biotinidase Antibody
Product Synonym Names
Biotinidase; Biotinase
Product Gene Name
anti-BTD antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P43251
Specificity
Recognizes endogenous levels of Biotinidase protein.
Purity/Purification
The antibody was purified by immunogen affinity chromatography.
Form/Format
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
Immunogen
Recombinant full length protein of human Biotinidase
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
Other Notes
Small volumes of anti-BTD antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-BTD antibody
Rabbit polyclonal antibody to Biotinidase
Applications Tested/Suitable for anti-BTD antibody
Western Blot (WB), Immunohistochemistry (IHC)
Application Notes for anti-BTD antibody
WB: 1/500 - 1/2000; IHC: 1/50 - 1/200
Western Blot (WB) of anti-BTD antibody
Western blot analysis of Biotinidase expression in mouse liver (A), mouse kidney (B) whole cell lysates.
Immunohistochemistry (IHC) of anti-BTD antibody
Immunohistochemical analysis of Biotinidase staining in rat brain formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
NCBI/Uniprot data below describe general gene information for BTD. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000051.1
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NCBI GenBank Nucleotide #
NM_000060.3
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UniProt Primary Accession #
P43251
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UniProt Secondary Accession #
Q96EM9; A6NHF2; B2R865; B4DFX1; B4DLJ9; B7Z7C9; F8W1Q3[Other Products]
UniProt Related Accession #
P43251[Other Products]
Molecular Weight
58,913 Da
NCBI Official Full Name
biotinidase isoform 3
NCBI Official Synonym Full Names
biotinidase
NCBI Official Symbol
BTD [Similar Products]
NCBI Protein Information
biotinidase
UniProt Protein Name
Biotinidase
Protein Family
Biotinidase
UniProt Gene Name
BTD [Similar Products]
UniProt Synonym Gene Names
Biotinase [Similar Products]
UniProt Entry Name
BTD_HUMAN
NCBI Summary for BTD
The protein encoded by this gene functions to recycle protein-bound biotin by cleaving biocytin (biotin-epsilon-lysine), a normal product of carboxylase degradation, resulting in regeneration of free biotin. The encoded protein has also been shown to have biotinyl transferase activity. Mutations in this gene are associated with biotinidase deficiency. Multiple transcript variants encoding different isoforms have been described. [provided by RefSeq, Aug 2013]
UniProt Comments for BTD
BTD: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation. Defects in BTD are the cause of biotinidase deficiency (BTD deficiency); also called late-onset multiple carboxylase deficiency. BTD deficiency is 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. BTD 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. Belongs to the CN hydrolase family. BTD/VNN subfamily.
Protein type: Secreted, signal peptide; Secreted; EC 3.5.1.12; Cofactor and Vitamin Metabolism - biotin; Hydrolase; Ligase
Chromosomal Location of Human Ortholog: 3p25
Cellular Component: extracellular region; extracellular space; mitochondrial matrix
Molecular Function: biotinidase activity; hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds
Biological Process: biotin metabolic process; central nervous system development
Disease: Biotinidase Deficiency
Research Articles on BTD
1. Summary of the demographic features of patients identified as biotinidase deficient from August of 2012 through August of 2013 and mutation analysis results for 20 cases in the southeast region of Turkey.
Precautions
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Disclaimer
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