Overview

  • Product name

  • Description

    Rabbit polyclonal to P5CS
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, IHC-Pmore details
  • Species reactivity

    Reacts with: Human
    Predicted to work with: Mouse, Rat
  • Immunogen

    Recombinant fragment, corresponding to amino acids 10-257 of Human P5CS (BC117240).

  • Positive control

    • Human fetal kidney, liver and spleen lysates.

Properties

Applications

Our Abpromise guarantee covers the use of ab111977 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/500 - 1/1000. Predicted molecular weight: 87 kDa.
IHC-P 1/100 - 1/500.

Target

  • Pathway

    Amino-acid biosynthesis; L-proline biosynthesis; L-glutamate 5-semialdehyde from L-glutamate: step 1/2.
    Amino-acid biosynthesis; L-proline biosynthesis; L-glutamate 5-semialdehyde from L-glutamate: step 2/2.
  • Involvement in disease

    Defects in ALDH18A1 are the cause of mental retardation-joint hypermobility-skin laxity with or without metabolic abnormalities (MRJHSL) [MIM:612652]. Clinical manifestations include microcephaly, progressive neurologic dysfunction, mental retardation, progeroid appearance, joint hypermobility, skin laxity and hyperelasticity, cataracts. Some patients manifest metabolic disturbances such as hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia.
  • Sequence similarities

    In the N-terminal section; belongs to the glutamate 5-kinase family.
    In the C-terminal section; belongs to the gamma-glutamyl phosphate reductase family.
  • Cellular localization

    Mitochondrion inner membrane.
  • Information by UniProt
  • Database links

  • Form

    P5CS catalyzes the ATP- and NADPH-dependent conversion of L-glutamate to glutamic gamma-semialdehyde, which is the metabolic precursor for proline biosynthesis. There are 2 isoforms produced by alternative splicing.
  • Alternative names

    • 2810433K04Rik antibody
    • AI429789 antibody
    • Aldehyde dehydrogenase 18 family member A1 antibody
    • Aldehyde dehydrogenase 18A1 antibody
    • Aldehyde dehydrogenase family 18 member A1 antibody
    • ALDH18A1 antibody
    • Delta 1 pyrroline 5 carboxylate synthetase antibody
    • Delta1 pyrroline 5 carboxlate synthetase antibody
    • Gamma-glutamyl kinase antibody
    • Gamma-glutamyl phosphate reductase antibody
    • GK antibody
    • Glutamate-5-semialdehyde dehydrogenase antibody
    • Glutamyl-gamma-semialdehyde dehydrogenase antibody
    • GPR antibody
    • GSAS antibody
    • MGC117316 antibody
    • MGC32233 antibody
    • P5CS antibody
    • P5CS_HUMAN antibody
    • PYCS antibody
    • Pyrroline 5 carboxylate synthetase (glutamate gamma semialdehyde synthetase) antibody
    see all

Images

  • All lanes : Anti-P5CS antibody (ab111977) at 1/500 dilution

    Lane 1 : Human fetal kidney lysate
    Lane 2 : Human fetal liver lysate
    Lane 3 : Human fetal spleen lysate

    Predicted band size: 87 kDa

References

This product has been referenced in:

  • Fischer B  et al. Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1. Mol Genet Metab 112:310-6 (2014). Read more (PubMed: 24913064) »
See 1 Publication for this product

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