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anti-BTD antibody :: Rabbit biotinidase Polyclonal Antibody

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Catalog # MBS710636
Unit / Price
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  0.05 mL  /  $230 +1 FREE 8GB USB
  0.15 mL  /  $500 +1 FREE 8GB USB
anti-BTD antibody
Product Name

biotinidase (BTD), Polyclonal Antibody

Full Product Name

Rabbit anti-human biotinidase polyclonal Antibody

Product Synonym Names
biotinidase;
Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
609019
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Antigen Affinity Purified
Immunogen
Human BTD
Storage Buffer
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
Santa Cruz Alternative
Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-102344 / sc-102346
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
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.
Applications Tested/Suitable for anti-BTD antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
NCBI/Uniprot data below describe general gene information for BTD. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
58,913 Da[Similar Products]
NCBI Official Full Name
biotinidase
NCBI Official Synonym Full Names
biotinidase
NCBI Official Symbol
NCBI Protein Information
biotinidase; biotinase
UniProt Protein Name
Biotinidase
Protein Family
UniProt Gene Name
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; Cofactor and Vitamin Metabolism - biotin; Ligase; EC 3.5.1.12; Secreted; Hydrolase

Chromosomal Location of Human Ortholog: 3p25

Cellular Component: extracellular space; mitochondrial matrix; apical part of cell; extracellular region; nucleolus; perikaryon

Molecular Function: biotinidase activity; biotin carboxylase activity

Biological Process: epidermis development; central nervous system development; vitamin metabolic process; water-soluble vitamin metabolic process; biotin metabolic process

Disease: Biotinidase Deficiency
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Products associated with anti-BTD antibody
 Reference Product  PubMed Publications
 HLCS antibody  >1 publications with BTD and HLCS
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