Anti-Human GLB1 Antibody Pair

Product Description: Galactosidase beta 1 (GLB1) is a lysosomal enzyme encoded by the GLB1 gene, primarily responsible for breaking down GM1 gangliosides and keratan sulfate, which are critical for neuronal function and connective tissue integrity. It cleaves terminal beta-galactose residues from glycoconjugates, enabling cellular recycling processes. Additionally, GLB1 encodes the elastin-binding protein, which forms part of the elastin receptor complex involved in elastic fiber assembly in connective tissues. This dual functionality underscores its importance in both metabolic and structural cellular roles. Mutations in GLB1 can lead to lysosomal storage disorders such as GM1 gangliosidosis and Morquio syndrome type B. These conditions result from impaired enzymatic activity, causing toxic accumulation of substrates like GM1 gangliosides, leading to neurodegeneration, skeletal abnormalities, and other systemic symptoms. Protein Function: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans , Has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non-integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers [The Uniprot Consortium]