Recombinant人BTD protein (ab116883)

概述

  • 产品名称Recombinant人BTD protein
  • 蛋白长度Full length protein

描述

  • 性质Recombinant
  • 来源Wheat germ
  • 氨基酸序列
    • AccessionP43251
    • 种属Human
    • 序列MAHAHIQGGRRAKSRFVVCIMSGARSKLALFLCGCYVVALGAHTGEESVA DHHEAEYYVAAVYEHPSILSLNPLALISRQEALELMNQNLDIYEQQVMTA AQKDVQIIVFPEDGIHGFNFTRTSIYPFLDFMPSPQVVRWNPCLEPHRFN DTEVLQRLSCMAIRGDMFLVANLGTKEPCHSSDPRCPKDGRYQFNTNVVF SNNGTLVDRYRKHNLYFEAAFDVPLKVDLITFDTPFAGRFGIFTCFDILF FDPAIRVLRDYKVKHVVYPTAWMNQLPLLAAIEIQKAFAVAFGINVLAAN VHHPVLGMTGSGIHTPLESFWYHDMENPKSHLIIAQVAKNPVGLIGAENA TGETDPSHSKFLKILSGDPYCEKDAQEVHCDEATKWNVNAPPTFHSEMMY DNFTLVPVWGKEGYLHVCSNGLCCYLLYERPTLSKELYALGVFDGLHTVH GTYYIQVCALVRCGGLGFDTCGQEITEATGIFEFHLWGNFSTSYIFPLFL TSGMTLEVPDQLGWENDHYFLRKSRLSSGLVTAALYGRLYERD
    • 分子量85 kDa including tags
    • 氨基酸1 to 543

相关产品

技术指标

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.

  • 应用

    ELISA

    SDS-PAGE

    Western blot

  • 形式Liquid
  • 补充说明Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • Biotinase
    • Biotinidase
    • Btd
    • BTD_HUMAN
    • EC 3.5.1.12
    see all
  • 功能Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
  • 疾病相关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.
  • 序列相似性Belongs to the CN hydrolase family. BTD/VNN subfamily.
    Contains 1 CN hydrolase domain.
  • 细胞定位Secreted > extracellular space.
  • Information by UniProt

Recombinant Human BTD protein 图像

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

Recombinant Human BTD protein (ab116883)参考文献

ab116883 has not yet been referenced specifically in any publications.

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