Overview

  • Product nameRecombinant Human BTD protein
  • Protein lengthFull length protein

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionP43251
    • SpeciesHuman
    • SequenceMAHAHIQGGRRAKSRFVVCIMSGARSKLALFLCGCYVVALGAHTGEESVA DHHEAEYYVAAVYEHPSILSLNPLALISRQEALELMNQNLDIYEQQVMTA AQKDVQIIVFPEDGIHGFNFTRTSIYPFLDFMPSPQVVRWNPCLEPHRFN DTEVLQRLSCMAIRGDMFLVANLGTKEPCHSSDPRCPKDGRYQFNTNVVF SNNGTLVDRYRKHNLYFEAAFDVPLKVDLITFDTPFAGRFGIFTCFDILF FDPAIRVLRDYKVKHVVYPTAWMNQLPLLAAIEIQKAFAVAFGINVLAAN VHHPVLGMTGSGIHTPLESFWYHDMENPKSHLIIAQVAKNPVGLIGAENA TGETDPSHSKFLKILSGDPYCEKDAQEVHCDEATKWNVNAPPTFHSEMMY DNFTLVPVWGKEGYLHVCSNGLCCYLLYERPTLSKELYALGVFDGLHTVH GTYYIQVCALVRCGGLGFDTCGQEITEATGIFEFHLWGNFSTSYIFPLFL TSGMTLEVPDQLGWENDHYFLRKSRLSSGLVTAALYGRLYERD
    • Molecular weight85 kDa including tags
    • Amino acids1 to 543

Associated products

Specifications

Our Abpromise guarantee covers the use of ab116883 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • FormLiquid
  • Additional notesProtein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.3% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • Biotinase
    • Biotinidase
    • Btd
    • BTD_HUMAN
    • EC 3.5.1.12
    see all
  • 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

Recombinant Human BTD protein images

  • 12.5% SDS-PAGE showing ab116883 at approximately 85.47kDa stained with Coomassie Blue.

References for Recombinant Human BTD protein (ab116883)

ab116883 has not yet been referenced specifically in any publications.

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