Key features and details
- Rabbit polyclonal to BTD
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-BTD antibody
See all BTD primary antibodies
DescriptionRabbit polyclonal to BTD
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse
Recombinant fragment, corresponding to a region within amino acids 206-449 of Human BTD (P43251).
- 293T, A431, Jurkat and Raji whole cell lysates.
General notesStore as concentrated solution.
Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.
Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.
We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.
In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 78.99% PBS, 1% BSA, 20% Glycerol (glycerin, glycerine)
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab126140 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/3000. Predicted molecular weight: 61 kDa.|
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
ab126140 has not yet been referenced specifically in any publications.