Key features and details
- Goat polyclonal to BTD
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid repeated freeze / thaw cycles.
Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: 99% Tris buffered saline, 0.5% BSA
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab166934 is purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Our Abpromise guarantee covers the use of ab166934 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 0.5 - 1.5 µg/ml. Predicted molecular weight: 61 kDa.
1 hour primary incubation is recommended for this product.
FunctionCatalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
Involvement in diseaseDefects in BTD are the cause of biotinidase deficiency (BTD deficiency) [MIM:253260]; 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.
Sequence similaritiesBelongs to the CN hydrolase family. BTD/VNN subfamily.
Contains 1 CN hydrolase domain.
Cellular localizationSecreted > extracellular space.
- Information by UniProt
- Biotinase antibody
- Biotinidase antibody
- Btd antibody
ab166934 has not yet been referenced specifically in any publications.