Recombinant Human GLB1 protein, His-tagged

• Cat.No.: GLB1-7758H
• Product Overview: Recombinant Human GLB1(Leu24-Val677) fused with His tag at C-terminal was expressed in HEK293.

Specification

• Description: β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases.
• Source: HEK293
• Species: Human
• Tag: His
• Form: Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.
• AA Sequence: LRNATQRMFEIDYSRDSFLKDGQPF RYISGSIHYSRVPRFYWKDRLLKMK MAGLNAIQTYVPWNF HEPWPGQYQFSEDHDVEYFLRLAHE LGLLVILRPGPYICAEWEMGGLPAW LLEKESILLRSSDPD YLAAVDKWLGVLLPKMKPLLYQNGG PVITVQVENEYGSYFACDFDYLRFL QKRFRHHLGDDVVLF TTDGAHKTFLKCGALQGLYTTVDFG TGSNITDAFLSQRKCEPKGPLINSE FYTGWLDHWGQPHST IKTEAVASSLYDILARGASVNLYMF IGGTNFAYWNGANSPYAAQPTSYDY DAPLSEAGDLTEKYF ALRNIIQKFEKVPEGPIPPSTPKFA YGKVTLEKLKTVGAALDILCPSGPI KSLYPLTFIQVKQHY GFVLYRTTLPQDCSNPAPLSSPLNG VHDRAYVAVDGIPQGVLERNNVITL NITGKAGATLDLLVE NMGRVNYGAYINDFKGLVSNLTLSS NILTDWTIFPLDTEDAVRSHLGGWG HRDSGHHDEAWAHNS SNYTLPAFYMGNFSIPSGIPDLPQD TFIQFPGWTKGQVWINGFNLGRYWP ARGPQLTLFVPQHIL MTSAPNTITVLELEWAPCSSDDPEL CAVTFVDRPVIGSSVTYDHPSKPVE KRLMPPPPQKNKDSW LDHVVDHHHHHH
• Endotoxin: Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test.
• Purity: Greater than 95% as determined by reducing SDS-PAGE.
• Storage: Store at < -20 centigrade, stable for 6 months after receipt.
  Please minimize freeze-thaw cycles.

Gene Information

• Gene Name: GLB1 galactosidase, beta 1 [ Homo sapiens ]
• Official Symbol: GLB1
• Synonyms: GLB1; galactosidase, beta 1; elastin receptor 1 (67kD) , elastin receptor 1, 67kDa , ELNR1; beta-galactosidase; EBP; lactase; acid beta-galactosidase; elastin receptor 1, 67kDa; ELNR1; MPS4B
• Gene ID: 2720
• mRNA Refseq: NM_000404
• Protein Refseq: NP_000395
• MIM: 611458
• UniProt ID: P16278
• Chromosome Location: 3p22.3
• Pathway: Galactose metabolism, organism-specific biosystem; Galactose metabolism, conserved biosystem; Glycosaminoglycan degradation, organism-specific biosystem; Glycosaminoglycan degradation, conserved biosystem; Glycosphingolipid biosynthesis - ganglio series, organism-specific biosystem; Glycosphingolipid biosynthesis - ganglio series, conserved biosystem; Glycosphingolipid metabolism, organism-specific biosystem
• Function: beta-galactosidase activity; cation binding; galactoside binding; hydrolase activity, hydrolyzing O-glycosyl compounds; protein binding