製品の概要

製品の詳細

  • 由来Recombinant
  • 由来Escherichia coli
  • アミノ酸配列
    • アクセッション番号P06132
    • 生物種Human
    • 配列MGSSHHHHHH SSGLVPRGSH MEANGLGPQG FPELKNDTFL RAAWGEETDY TPVWCMRQAG RYLPEFRETR AAQDFFSTCR SPEACCELTL QPLRRFPLDA AIIFSDILVV PQALGMEVTM VPGKGPSFPE PLREEQDLER LRDPEVVASE LGYVFQAITL TRQRLAGRVP LIGFAGAPWT LMTYMVEGGG SSTMAQAKRW LYQRPQASHQ LLRILTDALV PYLVGQVVAG AQALQLFESH AGHLGPQLFN KFALPYIRDV AKQVKARLRE AGLAPVPMII FAKDGHFALE ELAQAGYEVV GLDWTVAPKK ARECVGKTVT LQVNLDPCAL YASEEEIGQL VKQMLDDFGP HRYIANLGHG LYPDMDPEHV GAFVDAVHKH SRLLRQN
    • 分子量43 kDa including tags
    • 領域1 to 367
    • タグHis tag N-Terminus

特性

Our Abpromise guarantee covers the use of ab116491 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

    SDS-PAGE

  • 精製度> 95 % SDS-PAGE.
    ab116491 was purified by proprietary chromatographic techniques and filter sterilized.
  • 製品の状態Liquid
  • 備考although stable at 4°C for 1 week, should be stored desiccated below -18°C. Please prevent freeze thaw cycles.
  • Concentration information loading...

前処理および保存

  • 保存方法および安定性

    Shipped at 4°C. Please see notes section.

    pH: 8.00
    Constituents: 0.32% Tris HCl, 0.02% DTT, 0.58% Sodium chloride, 0.03% EDTA, 20% Glycerol

関連情報

  • 別名
    • DCUP_HUMAN
    • PCT
    • UPD
    • URO D
    • URO-D
    • urod
    • Uroporphyrinogen decarboxylase
    • Uroporphyrinogen III decarboxylase
    see all
  • 機能Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
  • パスウェイPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 4/4.
  • 関連疾患Defects in UROD are the cause of familial porphyria cutanea tarda (FPCT) [MIM:176100]; also known as porphyria cutanea tarda type II. FPCT is an autosomal dominant disorder characterized by light-sensitive dermatitis, with onset in later life. It is associated with the excretion of large amounts of uroporphyrin in the urine. Iron overload is often present in association with varying degrees of liver damage. Besides the familial form of PCT, a relatively common idiosyncratic form is known in which only the liver enzyme is reduced. This form is referred to as porphyria cutanea tarda "sporadic" type or type I [MIM:176090]. PCT type I occurs sporadically as an unusual accompaniment of common hepatic disorders such as alcohol-associated liver disease.
    Defects in UROD are the cause of hepatoerythropoietic porphyria (HEP) [MIM:176100]. HEP is a rare autosomal recessive disorder. It is the severe form of cutaneous porphyria, and presents in infancy. The level of UROD is very low in erythrocytes and cultured skin fibroblasts, suggesting that HEP is the homozygous state for porphyria cutanea tarda.
  • 配列類似性Belongs to the uroporphyrinogen decarboxylase family.
  • 細胞内局在Cytoplasm.
  • Information by UniProt

Recombinant Human UROD protein (ab116491) 使用論文

ab116491 has not yet been referenced specifically in any publications.

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