|Application ||WB, E|
|Calculated MW||91918 Da|
|Antigen Region||1-30 aa|
|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.|
|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.|
|Storage||Maintain 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.|
|Precautions||GCS1 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|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|
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Provided below are standard protocols that you may find useful for product applications.
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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