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GCS1 Antibody (C-term)

Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - GCS1 Antibody (C-term) AP2315b
    Western blot analysis of anti-GCS1 Pab (Cat. #AP2315b) in mouse liver cell line lysate (35ug/lane). GCS1(arrow) was detected using the purified Pab.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, E
Primary Accession Q13724
Other Accession NP_006293
Reactivity Human, Mouse
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Calculated MW 91918 Da
Antigen Region 796-826 aa
Additional Information
Gene ID 7841
Other Names Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I, MOGS, GCS1
Target/Specificity This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 796-826 amino acids from the C-terminal region of human GCS1.
Dilution WB~~1:1000
E~~Use at an assay dependent concentration.
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsGCS1 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name MOGS (HGNC:24862)
Function In the context of N-glycan degradation, cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.
Cellular Location Endoplasmic reticulum membrane; Single-pass type II membrane protein {ECO:0000250|UniProtKB:O88941}
Research Areas

For Research Use Only. Not For Use In Diagnostic Procedures.

BACKGROUND

GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and 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 include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.

REFERENCES

Volker, C., et al., Glycobiology 12(8):473-483 (2002).
De Praeter, C.M., et al., Am. J. Hum. Genet. 66(6):1744-1756 (2000).
Kalz-Fuller, B., et al., Eur. J. Biochem. 231(2):344-351 (1995).
Kalz-Fueller, B., et al., Eur. J. Biochem. 249, 912-912 (1997).

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