Glucosidase I Antibody (H-6): sc-365399

Glucosidase I Antibody (H-6) is a mouse monoclonal IgG1 antibody that detects Glucosidase I in mouse, rat, and human samples through various applications including western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), immunohistochemistry with paraffin-embedded sections (IHCP), and enzyme-linked immunosorbent assay (ELISA). Glucosidase I plays a crucial role in the N-linked glycosylation pathway, specifically catalyzing the removal of the distal alpha 1,2-linked glucose residue from the Glc3-Man9-GlcNAc2 oligosaccharide precursor within the lumen of the endoplasmic reticulum. This enzymatic activity is vital for proper protein folding and maturation, ensuring glycoproteins acquire the correct oligosaccharide structures necessary for stability and function. Disruptions in Glucosidase I activity due to genetic mutations can lead to congenital disorders of glycosylation, such as CDG-IIb, which manifest in severe clinical symptoms including hypotonia, dysmorphic features, and life-threatening complications. The structural integrity of Glucosidase I, characterized by a short cytosolic tail, single-pass transmembrane domain, and large C-terminal catalytic domain, is essential for function, as alterations can significantly impact glycoprotein processing. Glucosidase I (H-6) antibody serves as an invaluable tool for researchers studying glycosylation processes and related disorders.

The preferred Glucosidase I antibody is Glucosidase I Antibody (C-11): sc-374006.