Overview

  • Product name

  • Description

    Rabbit polyclonal to BTD
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 206-449 of Human BTD (P43251).

  • Positive control

    • 293T, A431, Jurkat and Raji whole cell lysates.
  • General notes

    Store as concentrated solution.

Properties

Applications

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.

Application Abreviews Notes
WB 1/500 - 1/3000. Predicted molecular weight: 61 kDa.

Target

  • Function

    Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
  • Involvement in disease

    Defects 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 similarities

    Belongs to the CN hydrolase family. BTD/VNN subfamily.
    Contains 1 CN hydrolase domain.
  • Cellular localization

    Secreted > extracellular space.
  • Information by UniProt
  • Database links

  • Alternative names

    • Biotinase antibody
    • Biotinidase antibody
    • Btd antibody
    • BTD_HUMAN antibody
    • EC 3.5.1.12 antibody
    see all

Images

  • Anti-BTD antibody (ab126140) at 1/1000 dilution + Jurkat lysate at 30 µg

    Predicted band size: 61 kDa



    7.5% SDS PAGE

References

ab126140 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

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