Overview

  • Product name
  • Description
    Rabbit polyclonal to GCS1
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse
  • Immunogen

    Recombinant fragment, corresponding to a region within amino acids 136-355 of Human GCS1 (UniProt ID: Q13724).

  • Positive control
    • 293T, A431, H1299, HeLaS3, HepG2, MOLT4 and Raji whole cell lysates.

Properties

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • Storage buffer
    pH: 7,00
    Preservative: 0.01% Thimerosal (merthiolate)
    Constituents: 0.75% Glycine, 1.21% Tris, 10% Glycerol
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab155231 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 - 1/3000. Predicted molecular weight: 92 kDa.

Target

  • Function
    Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.
  • Pathway
    Glycan metabolism; N-glycan degradation.
  • Involvement in disease
    Defects in MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]; also known as glucosidase I deficiency. CDGIIb is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months.
  • Sequence similarities
    Belongs to the glycosyl hydrolase 63 family.
  • Cellular localization
    Endoplasmic reticulum membrane.
  • Information by UniProt
  • Database links
  • Alternative names
    • EC 3.2.1.106 antibody
    • glucosidase I antibody
    • Mannosyl oligosaccharide glucosidase antibody
    • Mannosyl-oligosaccharide glucosidase antibody
    • Mogs antibody
    • MOGS_HUMAN antibody
    • Processing A glucosidase I antibody
    • Processing A-glucosidase I antibody
    see all

Images

  • Anti-GCS1 antibody (ab155231) at 1/1000 dilution + H1299 whole cell lysate at 30 µg

    Predicted band size: 92 kDa



    7.5% SDS-PAGE.

References

ab155231 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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