免疫原
Synthesized peptide derived from the Internal region of human Galactosidase β.
特异性
Galactosidase β Polyclonal Antibody detects endogenous levels of Galactosidase β protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB: 1/500 - 1/2000. IHC-p: 1:100-1:300. ELISA: 1/10000.. IF 1:50-200
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. [provided by RefSeq, Nov 2015],
功能
catalytic activity:Hydrolysis of terminal non-reducing beta-D-galactose residues in beta-D-galactosides.,disease:Defects in GLB1 are the cause of GM1-gangliosidosis type 1 (GM1G1) [MIM:230500]; also known as infantile GM1-gangliosidosis. GM1-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM1 gangliosides, glycoproteins and keratan sulfate primarily in neurons of the central nervous system. GM1G1 is characterized by onset within the first three months of life, central nervous system degeneration, coarse facial features, hepatosplenomegaly, skeletal dysmorphology reminiscent of Hurler syndrome, and rapidly progressive psychomotor deterioration. Urinary oligosaccharide levels are high. It leads to death usually between the first and second year of life.,disease:Defects in GLB1 are the cause of GM1-gangliosidosis type 2 (GM1G2) [MIM:230600];
其他名称
GLB1; ELNR1; Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin receptor 1
Fields
>>Galactose metabolism;>>Other glycan degradation;>>Glycosaminoglycan degradation;>>Sphingolipid metabolism;>>Glycosphingolipid biosynthesis - ganglio series;>>Metabolic pathways;>>Lysosome
细胞定位
[Isoform 1]: Lysosome .; [Isoform 2]: Cytoplasm, perinuclear region . Localized to the perinuclear area of the cytoplasm but not to lysosomes. .
组织表达
Detected in placenta (at protein level) (PubMed:8383699). Detected in fibroblasts and testis (PubMed:2511208).
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