製品の概要

  • 製品名Recombinant Human BTD protein
  • タンパク質長Full length protein

製品の詳細

  • 由来Recombinant
  • 由来Wheat germ
  • アミノ酸配列
    • アクセッション番号P43251
    • 生物種Human
    • 配列MAHAHIQGGRRAKSRFVVCIMSGARSKLALFLCGCYVVALGAHTGEESVA DHHEAEYYVAAVYEHPSILSLNPLALISRQEALELMNQNLDIYEQQVMTA AQKDVQIIVFPEDGIHGFNFTRTSIYPFLDFMPSPQVVRWNPCLEPHRFN DTEVLQRLSCMAIRGDMFLVANLGTKEPCHSSDPRCPKDGRYQFNTNVVF SNNGTLVDRYRKHNLYFEAAFDVPLKVDLITFDTPFAGRFGIFTCFDILF FDPAIRVLRDYKVKHVVYPTAWMNQLPLLAAIEIQKAFAVAFGINVLAAN VHHPVLGMTGSGIHTPLESFWYHDMENPKSHLIIAQVAKNPVGLIGAENA TGETDPSHSKFLKILSGDPYCEKDAQEVHCDEATKWNVNAPPTFHSEMMY DNFTLVPVWGKEGYLHVCSNGLCCYLLYERPTLSKELYALGVFDGLHTVH GTYYIQVCALVRCGGLGFDTCGQEITEATGIFEFHLWGNFSTSYIFPLFL TSGMTLEVPDQLGWENDHYFLRKSRLSSGLVTAALYGRLYERD
    • 分子量85 kDa including tags
    • 領域1 to 543

関連製品

特性

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.

  • アプリケーション

    ELISA

    SDS-PAGE

    Western blot

  • 製品の状態Liquid
  • 備考Protein concentration is above or equal to 0.05 mg/ml.
    Best used within three months from the date of receipt.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    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

関連情報

  • 別名
    • Biotinase
    • Biotinidase
    • Btd
    • BTD_HUMAN
    • EC 3.5.1.12
    see all
  • 機能Catalytic release of biotin from biocytin, the product of biotin-dependent carboxylases degradation.
  • 関連疾患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.
  • 配列類似性Belongs to the CN hydrolase family. BTD/VNN subfamily.
    Contains 1 CN hydrolase domain.
  • 細胞内局在Secreted > extracellular space.
  • Information by UniProt

Recombinant Human BTD protein 画像

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

Recombinant Human BTD protein (ab116883) 使用論文

ab116883 has not yet been referenced specifically in any publications.

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