|Other Names||Beta-galactosidase, Acid beta-galactosidase, Lactase, Elastin receptor 1, GLB1, ELNR1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Isoform 1: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans.|
|Cellular Location||Isoform 1: Lysosome|
|Tissue Location||Detected in placenta (at protein level) (PubMed:8383699). Detected in fibroblasts and testis (PubMed:2511208).|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes beta-galactosidase-1, a lysosomal enzymethat hydrolyzes the terminal beta-galactose from gangliosidesubstrates and other glycoconjugates. Defects in this gene are thecause of GM1-gangliosidosis and Morquio B syndrome. Multipletranscript variants encoding different isoforms have been found forthis gene.
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :Dubois, P.C., et al. Nat. Genet. 42(4):295-302(2010)Antonicelli, F., et al. Wound Repair Regen 17(5):631-638(2009)Hofer, D., et al. Hum. Mutat. 30(8):1214-1221(2009)Mayer, F.Q., et al. Mol. Genet. Metab. 96 (3), 148 (2009) :
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