Recombinant人ASS1 protein (ab116472)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • AccessionP00966
    • 种属Human
    • 序列MGSSHHHHHHSSGLVPRGSHMSSKGSVVLAYSGGLDTSCILVWLKEQGYD VIAYLANIGQKEDFEEARKKALKLGAKKVFIEDVSREFVEEFIWPAIQSS ALYEDRYLLGTSLARPCIARKQVEIAQREGAKYVSHGATGKGNDQVRFEL SCYSLAPQIKVIAPWRMPEFYNRFKGRNDLMEYAKQHGIPIPVTPKNPWS MDENLMHISYEAGILENPKNQAPPGLYTKTQDPAKAPNTPDILEIEFKKG VPVKVTNVKDGTTHQTSLELFMYLNEVAGKHGVGRIDIVENRFIGMKSRG IYETPAGTILYHAHLDIEAFTMDREVRKIKQGLGLKFAELVYTGFWHSPE CEFVRHCIAKSQERVEGKVQVSVLKGQVYILGRESPLSLYNEELVSMNVQ GDYEPTDATGFININSLRLKEYHRLQSKVTAK
    • 分子量49 kDa including tags
    • 氨基酸1 to 412
    • 标签His tag N-Terminus

技术指标

Our Abpromise guarantee covers the use of ab116472 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> 90 % SDS-PAGE.
    ab116472 is purified by proprietary chromatographic techniques.
  • 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, 0.58% Sodium chloride, 0.02% DTT, 20% Glycerol

常规信息

  • 别名
    • Argininosuccinate synthase
    • Argininosuccinate synthase 1
    • Argininosuccinate synthetase 1
    • ASS
    • Ass-1
    • ass1
    • ASSA
    • ASSY_HUMAN
    • Citrulline aspartate ligase
    • Citrulline--aspartate ligase
    • CTLN1
    see all
  • 通路Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 2/3.
    Nitrogen metabolism; urea cycle; (N(omega)-L-arginino)succinate from L-aspartate and L-citrulline: step 1/1.
  • 疾病相关Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1) [MIM:215700]. Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood.
  • 序列相似性Belongs to the argininosuccinate synthase family. Type 1 subfamily.
  • Information by UniProt

Recombinant Human ASS1 protein (ab116472)参考文献

ab116472 has not yet been referenced specifically in any publications.

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