Recombinant人UROD protein (ab116491)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionP06132
    • 种属Human
    • 序列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
    • 氨基酸1 to 367
    • 标签His tag N-Terminus

技术指标

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.

  • 应用

    SDS-PAGE

  • 纯度> 95 % SDS-PAGE.
    ab116491 was purified by proprietary chromatographic techniques and filter sterilized.
  • 形式Liquid
  • 补充说明although stable at 4°C for 1 week, should be stored desiccated below -18°C. Please prevent freeze thaw cycles.
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    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

常规信息

  • 别名
    • DCUP_HUMAN
    • PCT
    • UPD
    • URO D
    • URO-D
    • urod
    • Uroporphyrinogen decarboxylase
    • Uroporphyrinogen III decarboxylase
    see all
  • 功能Catalyzes the decarboxylation of four acetate groups of uroporphyrinogen-III to yield coproporphyrinogen-III.
  • 通路Porphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 4/4.
  • 疾病相关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.
  • 序列相似性Belongs to the uroporphyrinogen decarboxylase family.
  • 细胞定位Cytoplasm.
  • Information by UniProt

Recombinant Human UROD protein (ab116491)参考文献

ab116491 has not yet been referenced specifically in any publications.

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