抗体

β半乳糖苷酶抗体

MBS531235

1毫克

330美元

单克隆

小鼠

未共轭

2.00E+09

抗体

β半乳糖苷酶抗体

β半乳糖苷酶

Monoclonal beta Galactosidase; Anti-beta Galactosidase; B-Galactosidase; beta-Galactosidase; BGAL; B-Gal

beta-galactosidase preproprotein; Beta-galactosidase; beta-galactosidase; galactosidase, beta 1; Acid beta-galactosidase; Lactase

Glb1; Glb1; Bge; Bgl; Bgs; Bgt; Bgl-e; Bgl-s; Bgl-t; AW125515; C130097A14Rik; Bgl; Glb-1; Lactase

BGAL_MOUSE

单克隆

IgG1

2.00E+09

小鼠

EColi

647

Beta Galactosidase antibody was purified by chromatography on protein A Sepharose.

PBS, pH值7.4, 0.1% NaN3

Store at 4 degree C.

User optimized

Biological Significance: Beta-galactosidase is a hydrolase enzyme that catalyzes the hydrolysis of Beta-galactosides into monosaccharides. Substrates of different Beta-galactosidases include ganglioside GM1, lactosylceramides, lactose, and various glycoproteins.

Biohazard Information: This product contains sodium azide as preservative. Although the amount of sodium azide is very small appropriate care must be taken when handling this product. Immunogen: Beta galactosidase antibody was raised in mouse using purified b-galactosidase of E Coli as the immunogen.

Proteases, Inhibitors, & Enzymes

小鼠单克隆β半乳糖苷酶抗体

6753190

NP_033882.1

NM_009752.1

P23780

73,121 Da

Asparagine N-linked Glycosylation Pathway (1168804); Biosynthesis Of The N-glycan Precursor (dolichol Lipid-linked Oligosaccharide, LLO) And Transfer To A Nascent Protein Pathway (1168805); Galactose Metabolism Pathway (83131); Galactose Metabolism Pathway (292); Glycosaminoglycan Degradation Pathway (83180); Glycosaminoglycan Degradation Pathway (355); Glycosaminoglycan Metabolism Pathway (1168480); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (83196); Glycosphingolipid Biosynthesis - Ganglio Series Pathway (372); Glycosphingolipid Metabolism Pathway (1168595)

This gene encodes a preproprotein that is proteolytically cleaved to yield a signal peptide and a proproptein that is subsequently processed to generate the active mature peptide. The encoded protein is a lysosomal enzyme that catalyzes the hydrolysis of terminal beta-D-galactose residues in various substrates like lactose, ganglioside GM1 and other glycoproteins. Mutations in the human gene are associated with GM1-gangliosidosis and Morquio B syndrome. Disruption of the mouse gene mirrors the symptoms of human gangliosidosis. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2013]

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; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - other glycan degradation; Hydrolase; Lipid Metabolism - sphingolipid; EC 3.2.1.23. Cellular Component: Golgi apparatus; lysosome; cytoplasm; vacuole. Molecular Function: hydrolase activity; galactoside binding; hydrolase activity, acting on glycosyl bonds; beta-galactosidase activity; hydrolase activity, hydrolyzing O-glycosyl compounds. Biological Process: galactose catabolic process; metabolic process; carbohydrate metabolic process; cellular carbohydrate metabolic process

1毫克

330美元