Mannosyl-oligosaccharide glucosidase; Processing A-glucosidase I; MOGS; GCS1

For Research Use Only. Not for use in diagnostic procedures.

The synthetic peptide sequence used to generate the antibody was selected from the C-term region of human GCS1. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.

The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of MOGS blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner.

80,703 Da

mannosyl-oligosaccharide glucosidase

mannosyl-oligosaccharide glucosidase

Mannosyl-oligosaccharide glucosidase

GCS1??[Similar Products]

MOGS_HUMAN

This gene encodes the first enzyme in the N-linked oligosaccharide processing pathway. The enzyme cleaves the distal alpha-1,2-linked glucose residue from the Glc(3)-Man(9)-GlcNAc(2) oligosaccharide precursor. This protein is located in the lumen of the endoplasmic reticulum. Defects in this gene are a cause of type IIb congenital disorder of glycosylation (CDGIIb). Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]

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 MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb); 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. Belongs to the glycosyl hydrolase 63 family.

Protein type: Hydrolase; Endoplasmic reticulum; Glycan Metabolism - N-glycan biosynthesis; EC 3.2.1.106; Membrane protein, integral

Chromosomal Location of Human Ortholog: 2p13.1

Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; membrane

Molecular Function: glucosidase activity; mannosyl-oligosaccharide glucosidase activity

Biological Process: protein amino acid N-linked glycosylation; protein folding

Disease: Congenital Disorder Of Glycosylation, Type Iib

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