Galactosidase Beta (GLb) Recombinant Protein | GLb recombinant protein

Recombinant Galactosidase Beta (GLb)

Cat. Num. AAA2009828

• Gene Names: Glb1; Bge; Bgl; Bgs; Bgt; Bgl-e; Bgl-s; Bgl-t; AW125515; C130097A14Rik
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: > 95%
• Synonyms: Galactosidase Beta (GLb); Recombinant Galactosidase Beta (GLb); GLb recombinant protein

• Host: E Coli
• Purity/Purification: > 95%
• Form/Format: Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.
• Sequence: The target protein is fused with N-terminal His-Tag, its sequence is listed below.; MGHHHHHHSG SEF-SYFACDYDY LRFLVHRFRY HLGNDVILFT TDGASEKMLK CGTLQDLYAT VDFGTGNNIT QAFLVQRKFE PKGPLINSEF YTGWLDHWGK PHSTVKTKTL ATSLYNLLAR GANVNLYMFI GGTNFAYWNG ANTPYEPQPT SYDYDAPLSE AGDLTKKYFA LREVIQMFKE VPEGPIPPST PKFAYGKVAL RKFKTVAEAL GILCPNGPVK SLYPLTFTQV KQYFGYVLYR TTLPQDCSNP KP
• Sequence Length: 647

• Applicable Applications for GLb recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Organism: Mus musculus (Mouse)
• Expression System: Prokaryotic expression
• Residues: Ser192~Pro432 (Accession # P23780) with N-terminal His-Tag
• Subcellular Location: Lysosome
• Endotoxin Level: <1.0EU per 1ug (determined by the LAL method)
• Predicted Isoelectric Point: 8.7
• Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.
• Preparation and Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

SDS-Page

NCBI and Uniprot Product Information

• NCBI GI #: 6753190
• NCBI GeneID: 12091
• NCBI Accession #: NP_033882.1
• NCBI GenBank Nucleotide #: NM_009752.2
• UniProt Accession #: P23780
• Molecular Weight: 28.8kDa
• NCBI Official Full Name: beta-galactosidase preproprotein
• NCBI Official Synonym Full Names: galactosidase, beta 1
• NCBI Official Symbol: Glb1
• NCBI Official Synonym Symbols: Bge; Bgl; Bgs; Bgt; Bgl-e; Bgl-s; Bgl-t; AW125515; C130097A14Rik
• NCBI Protein Information: beta-galactosidase; lactase; acid beta-galactosidase
• UniProt Protein Name: Beta-galactosidase
• Protein Family: Nitrogen regulatory protein
• UniProt Gene Name: Glb1
• UniProt Synonym Gene Names: Bgl; Glb-1; Lactase
• UniProt Entry Name: BGAL_MOUSE

NCBI Description

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]

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

Cellular Component: Golgi apparatus; lysosome; cytoplasm; vacuole

Molecular Function: hydrolase activity; hydrolase activity, acting on glycosyl bonds; galactoside binding; beta-galactosidase activity; hydrolase activity, hydrolyzing O-glycosyl compounds

Biological Process: galactose catabolic process; metabolic process; carbohydrate metabolic process; cellular carbohydrate metabolic process