Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      MGSSHHHHHHSSGLVPRGSHMSSKGSVVLAYSGGLDTSCILVWLKEQGYD VIAYLANIGQKEDFEEARKKALKLGAKKVFIEDVSREFVEEFIWPAIQSS ALYEDRYLLGTSLARPCIARKQVEIAQREGAKYVSHGATGKGNDQVRFEL SCYSLAPQIKVIAPWRMPEFYNRFKGRNDLMEYAKQHGIPIPVTPKNPWS MDENLMHISYEAGILENPKNQAPPGLYTKTQDPAKAPNTPDILEIEFKKG VPVKVTNVKDGTTHQTSLELFMYLNEVAGKHGVGRIDIVENRFIGMKSRG IYETPAGTILYHAHLDIEAFTMDREVRKIKQGLGLKFAELVYTGFWHSPE CEFVRHCIAKSQERVEGKVQVSVLKGQVYILGRESPLSLYNEELVSMNVQ GDYEPTDATGFININSLRLKEYHRLQSKVTAK
    • Molecular weight
      49 kDa including tags
    • Amino acids
      1 to 412
    • Tags
      His tag N-Terminus

Specifications

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.

  • Applications

    SDS-PAGE

  • Purity
    > 90 % SDS-PAGE.
    ab116472 is purified by proprietary chromatographic techniques.
  • Form
    Liquid
  • Additional notes
    although stable at 4°C for 1 week, should be stored desiccated below -18°C. Please prevent freeze thaw cycles.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    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

General Info

  • Alternative names
    • Argininosuccinate synthase
    • Argininosuccinate synthase 1
    • Argininosuccinate synthetase 1
    • ASS
    • Ass-1
    • ass1
    • ASSA
    • ASSY_HUMAN
    • Citrulline aspartate ligase
    • Citrulline--aspartate ligase
    • CTLN1
    see all
  • Pathway
    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.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the argininosuccinate synthase family. Type 1 subfamily.
  • Information by UniProt

References

ab116472 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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