Human Mannosyl-oligosaccharide glucosidase (MOGS) ELISA kit; GCS1; glucosidase I; processing A-glucosidase I; mannosyl-oligosaccharide glucosidase

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

Store all reagents at 2-8 degree C

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Small volumes of MOGS elisa kit 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.

MBS9333305 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the mannosyl-oligosaccharide glucosidase (MOGS) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MOGS. The ELISA analytical biochemical technique of the MBS9333305 kit is based on MOGS antibody-MOGS antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MOGS antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MOGS. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

91,918 Da

mannosyl-oligosaccharide glucosidase

mannosyl-oligosaccharide glucosidase; glucosidase I; processing A-glucosidase I

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

Chromosomal Location of Human Ortholog: 2p13.1

Cellular Component: endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane

Molecular Function: mannosyl-oligosaccharide glucosidase activity; glucosidase activity

Biological Process: cellular protein metabolic process; protein folding; protein amino acid N-linked glycosylation; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification; oligosaccharide metabolic process

Disease: Congenital Disorder Of Glycosylation, Type Iib

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