Rabbit anti-Human BTD Polyclonal Antibody | anti-BTD antibody

BTD antibody

Cat. Num. AAA9412912

• Reactivity: Human
• Applications: Western Blot
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: BTD; Polyclonal Antibody; BTD antibody; anti-BTD antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total BTD antibody.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 543

• Applicable Applications for anti-BTD antibody: Western Blot (WB)
• Application Notes: Western blotting: 1:500 - 1:2000
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human BTD.
• Target Name: BTD
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of various tissues, using BTD antibody.)

Related Product Information for anti-BTD antibody

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.

Product Categories/Family for anti-BTD antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4557373
• NCBI GeneID: 686
• NCBI Accession #: NP_000051.1
• NCBI GenBank Nucleotide #: NM_000060.3
• UniProt Accession #: P43251; Q96EM9; A6NHF2; B2R865; B4DFX1; B4DLJ9; B7Z7C9; F8W1Q3
• Molecular Weight: 58,913 Da
• NCBI Official Full Name: biotinidase isoform 3
• NCBI Official Synonym Full Names: biotinidase
• NCBI Official Symbol: BTD
• NCBI Protein Information: biotinidase
• UniProt Protein Name: Biotinidase
• Protein Family: Biotinidase
• UniProt Gene Name: BTD
• UniProt Synonym Gene Names: Biotinase
• UniProt Entry Name: BTD_HUMAN

NCBI Description

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 Description

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; EC 3.5.1.12; Ligase; Cofactor and Vitamin Metabolism - biotin; 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; vitamin metabolic process; central nervous system development; biotin metabolic process; water-soluble vitamin metabolic process

Disease: Biotinidase Deficiency