Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MPSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKEPLWIRPDAPS RCTWQLGRPASESPHHHTAPAKSPKILPDILKKIGDTPMVRINKIGKKFG LKCELLAKCEFFNAGGSVKDRISLRMIEDAERDGTLKPGDTIIEPTSGNT GIGLALAAAVRGYRCIIVMPEKMSSEKVDVLRALGAEIVRTPTNARFDSP ESHVGVAWRLKNEIPNSHILDQYRNASNPLAHYDTTADEILQQCDGKLDM LVASVGTGGTITGIARKLKEKCPGCRIIGVDPEGSILAEPEELNQTEQTT YEVEGIGYDFIPTVLDRTVVDKWFKSNDEEAFTFARMLIAQEGLLCGGSA GSTVAVAVKAAQELQEGQRCVVILPDSVRNYMTKFLSDRWMLQKGFLKEE DLTEKKPWWWHLRVQELGLSAPLTVLPTITCGHTIEILREKGFDQAPVVD EAGVILGMVTLGNMLSSLLAGKVQPSDQVGKVIYKQFKQIRLTDTLGRLS HILEMDHFALVVHEQIQYHSTGKSSQRQMVFGVVTAIDLLNFVAAQERDQ K
    • Molecular weight

      86 kDa including tags
    • Amino acids

      1 to 551
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab132564 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • AI047524
    • AI303044
    • Beta thionase
    • Beta-thionase
    • Cbs
    • Cbs cystathionine beta-synthase
    • CBS_HUMAN
    • Cystathionine beta synthase
    • Cystathionine beta-synthase
    • EC 4.2.1.22
    • HIP 4
    • HIP4
    • Methylcysteine synthase
    • MGC18856
    • MGC18895
    • MGC37300
    • OTTHUMP00000109416
    • OTTHUMP00000109418
    • Serine sulfhydrase
    see all
  • Function

    Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury.
  • Tissue specificity

    In the adult strongly expressed in liver and pancreas, some expression in heart and brain, weak expression in lung and kidney. In the fetus, expressed in brain, liver and kidney.
  • Pathway

    Amino-acid biosynthesis; L-cysteine biosynthesis; L-cysteine from L-homocysteine and L-serine: step 1/2.
  • Involvement in disease

    Defects in CBS are the cause of cystathionine beta-synthase deficiency (CBSD) [MIM:236200]. CBSD is an enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine.
  • Sequence similarities

    Belongs to the cysteine synthase/cystathionine beta-synthase family.
    Contains 1 CBS domain.
  • Cellular localization

    Cytoplasm. Nucleus.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE analysis of ab132564 stained with Coomassie Blue.

References

ab132564 has not yet been referenced specifically in any publications.

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