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Anti-BTD Antibody

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产品名称: Anti-BTD Antibody
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简单介绍

Anti-BTD Antibody


Anti-BTD Antibody  的详细介绍
Name: Anti-BTD Antibody
See all BTD primary antibodies
Description: Goat polyclonal antibody to BTD.
Applications: ELISA, WB
Reactivity: Human
Immunogen: Synthetic peptide corresponding to Human BTD (internal region).
Sequence: EKDAQEVHCDEATK
Host: Goat
Clonality: Polyclonal
Isotype: IgG
Conjugate: Unconjugated
Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration: 100 µg at 0.5 mg/ml.
Product Form: Liquid
Formulation: Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Function: Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
Involvement in Disease: Biotinidase 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.
Sequence Similarities: Belongs to the carbon-nitrogen hydrolase superfamily. BTD/VNN family.
Cellular Location: Secreted > Extracellular space.
Database Links:
  • Entrez Gene: 686 Human
  • Omim: 609019 Human
  • SwissProt: P43251 Human
  • Unigene: 517830 Human
    		•
    Synonyms:
  • Biotinase Antibody
  • Biotinidase Antibody
  • BTD Antibody
  • BTD_HUMAN Antibody
  • EC 3.5.1.12 Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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