{"product_id":"nsj-fy13064-100ug","title":"GALC Antibody \/ Galactocerebrosidase, 100 ug","description":"\u003cp\u003eGALC antibody detects galactocerebrosidase, a lysosomal enzyme that catalyzes the hydrolysis of galactosylceramide and psychosine, both critical for normal myelin turnover. The UniProt recommended name is Galactocerebrosidase (GALC). This enzyme removes the galactose moiety from specific sphingolipids, supporting lipid recycling and the stability of central and peripheral nervous system myelin.\u003cbr\u003e\u003cbr\u003eFunctionally, GALC antibody recognizes a precursor glycoprotein that is proteolytically processed into 50 kDa and 30 kDa subunits forming the active heterodimeric enzyme within lysosomes. Galactocerebrosidase is essential for degrading galactolipids, and mutations in the GALC gene lead to Krabbe disease (globoid cell leukodystrophy), characterized by psychosine accumulation, oligodendrocyte death, and severe demyelination. The enzyme�s deficiency disrupts lipid metabolism, resulting in neuroinflammation and white matter loss.\u003cbr\u003e\u003cbr\u003eThe GALC gene, located on chromosome 14q31.3, encodes a lysosomal hydrolase expressed in oligodendrocytes, Schwann cells, and various other tissues involved in lipid degradation. The protein�s N-linked glycosylation and mannose-6-phosphate tagging ensure proper lysosomal trafficking. GALC activity is tightly regulated by substrate availability and cellular differentiation state, making it a key player in neuronal lipid homeostasis.\u003cbr\u003e\u003cbr\u003ePathologically, GALC dysfunction is the molecular hallmark of Krabbe disease, but altered expression has also been linked to other neurodegenerative and demyelinating disorders. Research using GALC antibody helps elucidate disease mechanisms and assess therapeutic strategies such as enzyme replacement or gene therapy. Immunodetection of GALC in cells or tissues serves as a biomarker for lysosomal integrity and treatment response.\u003cbr\u003e\u003cbr\u003eGALC antibody is suitable for western blotting, immunocytochemistry, and immunohistochemistry to visualize lysosomal distribution and measure enzyme levels in normal and pathological tissues. It supports investigations into lysosomal storage disorders, lipid catabolism, and neuronal repair. NSJ Bioreagents provides GALC antibody reagents validated for lysosomal enzyme and myelin biology research.\u003cbr\u003e\u003cbr\u003eStructurally, galactocerebrosidase contains a (beta\/alpha)8-barrel catalytic core typical of glycosidases. Disease-causing mutations cluster around active-site residues or disrupt domain folding, leading to reduced stability or mislocalization. Studies using this antibody contribute to understanding GALC's enzymology, trafficking, and role in myelin preservation.\u003c\/p\u003e","brand":"NSJ Bioreagents","offers":[{"title":"100 ug","offer_id":51552335921432,"sku":"FY13064","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/get_image_061ed3a3-3521-4eef-9116-5649ca8f8e4a.jpg?v=1768353051","url":"https:\/\/danabiosci.com\/products\/nsj-fy13064-100ug","provider":"Dana Bioscience","version":"1.0","type":"link"}