{"product_id":"nsj-fy13225-100ug","title":"POMT1 Antibody \/ Protein O-mannosyl-transferase 1, 100 ug","description":"\u003cp\u003ePOMT1 antibody detects Protein O-mannosyl-transferase 1, an enzyme required for the initial step of O-mannosyl glycan biosynthesis on glycoproteins, particularly alpha-dystroglycan. The UniProt recommended name is Protein O-mannosyl-transferase 1 (POMT1). This enzyme catalyzes the transfer of mannose from dolichol phosphate-mannose to serine or threonine residues on target proteins, a process essential for proper protein folding, stability, and function.\u003cbr\u003e\u003cbr\u003eFunctionally, POMT1 antibody identifies a 747-amino-acid endoplasmic reticulum (ER) membrane protein that forms a functional heterodimer with POMT2. This complex initiates the O-mannosyl glycosylation pathway, which is critical for dystroglycan processing and muscle integrity. POMT1 ensures the attachment of the first mannose residue, forming the foundation for subsequent glycan extensions that link the extracellular matrix to the cytoskeleton via alpha-dystroglycan.\u003cbr\u003e\u003cbr\u003eThe POMT1 gene is located on chromosome 9q34.13 and is expressed in brain, skeletal muscle, and heart. It plays a vital role in muscle development, neuronal migration, and synaptic organization by supporting glycosylation-dependent cell adhesion and signaling.\u003cbr\u003e\u003cbr\u003ePathologically, mutations in POMT1 cause congenital muscular dystrophy-dystroglycanopathy type A1 (Walker-Warburg syndrome), type B1, and type C1. These disorders are characterized by muscular dystrophy, brain malformations, and ocular defects due to defective O-mannosylation of dystroglycan. Research using POMT1 antibody supports studies in glycosylation pathways, muscular dystrophy, and neuronal development.\u003cbr\u003e\u003cbr\u003ePOMT1 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect ER glycosyltransferases. NSJ Bioreagents provides POMT1 antibody reagents optimized for studies in protein modification, muscle biology, and glycoprotein processing.\u003cbr\u003e\u003cbr\u003eStructurally, Protein O-mannosyl-transferase 1 contains multiple transmembrane domains and a luminal catalytic domain harboring the conserved DXD motif required for glycosyltransferase activity. It assembles with POMT2 to form an enzymatically active complex that determines substrate recognition and catalysis. This antibody aids in investigating POMT1�s role in glycoprotein biosynthesis, muscle development, and congenital disease mechanisms.\u003c\/p\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ug","offer_id":48537661341920,"sku":"FY13225","price":449.0,"currency_code":"KRW","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0601\/9330\/8896\/files\/get_image_928b72f7-e386-48fc-8ae1-7bddac5c2284.jpg?v=1768352734","url":"https:\/\/rndmate.com\/products\/nsj-fy13225-100ug","provider":"알앤디메이트","version":"1.0","type":"link"}