Western Blot (WB)
(Western blot analysis of Beta-galactosidase expression in HEK293T (A), HepG2 (B), PC12 (C) whole cell lysates.)
Rabbit anti-Human, Rat Beta-galactosidase Polyclonal Antibody | anti-GLB1 antibody
Anti-Beta-galactosidase Antibody
Western Blot (WB)
(Western blot analysis of Beta-galactosidase expression in HEK293T (A), HepG2 (B), PC12 (C) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of Beta-galactosidase expression in HEK293T (A), HepG2 (B), PC12 (C) whole cell lysates.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes beta-galactosidase-1, a lysosomal enzyme that hydrolyzes the terminal beta-galactose from ganglioside substrates and other glycoconjugates. Defects in this gene are the cause of GM1-gangliosidosis and Morquio B syndrome. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]
Uniprot Description
GLB1: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Defects in GLB1 are the cause of GM1-gangliosidosis type 1 (GM1G1); 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. Defects in GLB1 are the cause of GM1-gangliosidosis type 2 (GM1G2); also known as late infantile/juvenile GM1- gangliosidosis. GM1G2 is characterized by onset between ages 1 and 5. The main symptom is locomotor ataxia, ultimately leading to a state of decerebration with epileptic seizures. Patients do not display the skeletal changes associated with the infantile form, but they nonetheless excrete elevated amounts of beta-linked galactose-terminal oligosaccharides. Inheritance is autosomal recessive. Defects in GLB1 are the cause of GM1-gangliosidosis type 3 (GM1G3); also known as adult or chronic GM1- gangliosidosis. GM1G3 is characterized by a variable phenotype. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Intellectual deficit can initially be mild or absent but progresses over time. Inheritance is autosomal recessive. Defects in GLB1 are the cause of mucopolysaccharidosis type 4B (MPS4B); also known as Morquio syndrome B. MPS4B is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. Belongs to the glycosyl hydrolase 35 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Carbohydrate Metabolism - galactose; Hydrolase; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - other glycan degradation; Lipid Metabolism - sphingolipid; Glycan Metabolism - glycosaminoglycan degradation; EC 3.2.1.23
Chromosomal Location of Human Ortholog: 3p21.33
Cellular Component: Golgi apparatus; lysosomal lumen; perinuclear region of cytoplasm; cytoplasm; vacuole
Molecular Function: protein binding; galactoside binding; beta-galactosidase activity
Biological Process: keratan sulfate metabolic process; sphingolipid metabolic process; dolichol-linked oligosaccharide biosynthetic process; glycosaminoglycan metabolic process; pathogenesis; post-translational protein modification; galactose catabolic process; glycosaminoglycan catabolic process; cellular protein metabolic process; carbohydrate metabolic process; protein amino acid N-linked glycosylation via asparagine; cellular carbohydrate metabolic process; glycosphingolipid metabolic process; keratan sulfate catabolic process
Disease: Gm1-gangliosidosis, Type Iii; Mucopolysaccharidosis Type Ivb; Gm1-gangliosidosis, Type Ii; Gm1-gangliosidosis, Type I
Research Articles on GLB1
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Product Notes
The GLB1 glb1 (Catalog #AAA821326) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-Beta-galactosidase Antibody reacts with Human, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Beta-galactosidase can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB (1/500 - 1/1000). Researchers should empirically determine the suitability of the GLB1 glb1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Beta-galactosidase, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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