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GCS1 Antibody (N-term)

Purified Rabbit Polyclonal Antibody (Pab)

  • WB - GCS1 Antibody (N-term) AP2315a
    Western blot analysis of anti-GCS1 Pab (Cat. #AP2315a) in 293 cell line lysates (35ug/lane). GCS1 (arrow) was detected using the purified Pab.
Product Information
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immunoelectronmicroscopy
  • EIA=Enzyme Immunoassay
Primary Accession Q13724
Other Accession NP_006293
Reactivity Human
Host Rabbit
Clonality Polyclonal
Isotype Rabbit Ig
Calculated MW 91918 Da
Antigen Region 1-30 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 1-30 amino acids from the N-terminal region of human GCS1.
Dilution WB~~1:1000
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 (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Synonyms GCS1
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.
Cellular Location Endoplasmic reticulum membrane; Single-pass type II membrane protein
Citations (0)

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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.


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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$ 295.00
$ 99.00
Cat# AP2315a
(40 western blots)
Availability: In Stock
Bulk Size
Seasonal Special on Bulk Order
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