Molecular weight (DA)
76kD
Immunogen
Synthesized peptide derived from the Internal region of human Galactosidase β.
Specificity
Galactosidase β Polyclonal Antibody detects endogenous levels of Galactosidase β protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC-p: 1:100-1:300. ELISA: 1/10000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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],
Function
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];
Protein name
Beta-galactosidase
Abbreviation
Galactosidase β
Other name
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
Human protein sequence Database
P16278
Mouse protein sequence database
P23780
Rat protein sequence database
Cellular localization
[Isoform 1]: Lysosome .; [Isoform 2]: Cytoplasm, perinuclear region . Localized to the perinuclear area of the cytoplasm but not to lysosomes. .
Tissue expression
Detected in placenta (at protein level) (PubMed:8383699). Detected in fibroblasts and testis (PubMed:2511208).
Contract Us
Follow Us
Location： 
Specification
成功添加到购物车