Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • AccessionP00966
    • SpeciesHuman
    • SequenceMGSSHHHHHH SSGLVPRGSH MSSKGSVVLA YSGGLDTSCI LVWLKEQGYD VIAYLANIGQ KEDFEEARKK ALKLGAKKVF IEDVSREFVEEFIWPAIQSS ALYEDRYLLG TSLARPCIAR KQVEIAQREG AKYVSHGATG KGNDQVRFEL SCYSLAPQIK VIAPWRMPEF YNRFKGRNDL MEYAKQHGIP IPVTPKNPWS MDENLMHISY EAGILENPKN QAPPGLYTKT QDPAKAPNTP DILEIEFKKG VPVKVTNVKD GTTHQTSLELFMYLNEVAGK HGVGRIDIVE NRFIGMKSRG IYETPAGTIL YHAHLDIEAF TMDREVRKIK QGLGLKFAEL VYTGFWHSPE CEFVRHCIAK SQERVEGKVQ VSVLKGQVYI LGRESPLSLY NEELVSMNVQ GDYEPTDATG FININSLRLK EYHRLQSKVT AK
    • Molecular weight49 kDa including tags
    • Amino acids1 to 412
    • TagsHis tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab98084 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

  • Mass spectrometry
    MALDI-TOF
  • Purity> 90 % SDS-PAGE.
    ab98084 was purified using conventional chromatography techniques.
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.

    Preservative: None
    Constituents: 20% Glycerol, 0.1M Sodium chloride, 20mM Tris HCl, 0.1mM DTT, pH 8

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
  • PathwayAmino-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 diseaseDefects 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 similaritiesBelongs to the argininosuccinate synthase family. Type 1 subfamily.
  • Information by UniProt

Recombinant Human ASS1 protein images

  • 15% SDS-PAGE analysis of ab98084 (3µg)

References for Recombinant Human ASS1 protein (ab98084)

ab98084 has not yet been referenced specifically in any publications.

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