|Application ||WB, E|
|Other Accession||Q60HF6, NP_000395.2, NP_001073279.1|
|Calculated MW||76075 Da|
|Antigen Region||81-109 aa|
|Other Names||Beta-galactosidase, Acid beta-galactosidase, Lactase, Elastin receptor 1, GLB1, ELNR1|
|Target/Specificity||This GLB1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 81-109 amino acids from the N-terminal region of human GLB1.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||GLB1 Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans.|
|Cellular Location||Isoform 1: Lysosome.|
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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 enzyme that hydrolyzes the terminal beta-galactose from ganglioside substrates and other glycoconjugates. Defects in this gene are the cause of GM1-gangliosidosis and Morquio B syndrome. Multiple transcript variants encoding different isoforms have been found for this 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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