Rabbit anti-Human, Rat GLB1 Polyclonal Antibody | anti-GLB1 antibody

GLB1 Polyclonal Antibody

Cat. Num. AAA3006731

• Gene Names: GLB1; EBP; ELNR1; MPS4B
• Reactivity: Human, Rat
• Applications: Western Blot
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Synonyms: GLB1; Polyclonal Antibody; GLB1 Polyclonal Antibody; Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin receptor 1; ELNR1; anti-GLB1 antibody

• Host: Rabbit
• Reactivity: Human, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: GLB1 polyclonal antibody detects endogenous levels of GLB1 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is >95% (by SDS-PAGE).
• Form/Format: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
• Sequence Length: 677

• Applicable Applications for anti-GLB1 antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:1000
• Immunogen: A synthetic peptide corresponding to residues in Human GLB1.
• Preparation and Storage: Store at 4 degree C short term.; Aliquot and store at -20 degree C long term.; Avoid freeze-thaw cycles.

Western Blot (WB)

(Western Blot (WB) analysis of GLB1 polyclonal antibody at 1:500 dilutionLane1:HEK293T whole cell lysateLane2:HepG2 whole cell lysateLane3:PC12 whole cell lysate)

Related Product Information for anti-GLB1 antibody

The human b-galactosidase gene, known as the LacZ gene, maps to chromosome 3p21.33 and encodes a 677 amino acid protein with an optimum functional pH range of 6 to 8. Catalytically active b-galactosidases (b-Gal) is a tetramer of four identical subunits, each with an active site, which can independently catalyze the cleavage of terminal galactose. Monovalent cations have a stimulatory effect on the enzymatic reaction, which likely involves a galactosyl-enzyme complex intermediate. b-Gals are widespread in animals, microorganisms and plants. The LacZ gene is widely used as a reporter gene with a variety of colored or fluorescent compounds capable of being produced from appropriate substrates, such as Xgal, which produces a blue color. For this reason, LacZ is incorporated into numerous plasmid vectors as a marker.

NCBI and Uniprot Product Information

• NCBI GI #: 119372308
• NCBI GeneID: 2720
• NCBI Accession #: NP_000395.2
• NCBI GenBank Nucleotide #: NP_000395.2
• UniProt Accession #: P16278
• Molecular Weight: ~ 90kDa
• NCBI Official Full Name: beta-galactosidase isoform a preproprotein
• NCBI Official Synonym Full Names: galactosidase beta 1
• NCBI Official Symbol: GLB1
• NCBI Official Synonym Symbols: EBP; ELNR1; MPS4B
• NCBI Protein Information: beta-galactosidase
• UniProt Protein Name: Beta-galactosidase
• Protein Family: Globulin
• UniProt Gene Name: GLB1
• UniProt Synonym Gene Names: ELNR1; Lactase
• UniProt Entry Name: BGAL_HUMAN

NCBI Description

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]

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: Glycan Metabolism - other glycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosaminoglycan degradation; Lipid Metabolism - sphingolipid; EC 3.2.1.23; Carbohydrate Metabolism - galactose; Hydrolase

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; glycosphingolipid metabolic process; cellular carbohydrate metabolic process; keratan sulfate catabolic process

Disease: Gm1-gangliosidosis, Type Iii; Mucopolysaccharidosis Type Ivb; Gm1-gangliosidosis, Type Ii; Gm1-gangliosidosis, Type I