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

GLB1 (Beta-galactosidase, Acid beta-galactosidase, Lactase, Elastin Receptor 1, ELNR1) (PE)

Cat. Num. AAA6379856

• Gene Names: GLB1; EBP; ELNR1; MPS4B
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: GLB1; Polyclonal Antibody; GLB1 (Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin Receptor 1; ELNR1) (PE); anti-GLB1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human GLB1.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).
• Sequence Length: 677

• Applicable Applications for anti-GLB1 antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human GLB1, aa1-677 (AAH07493.1).
• Immunogen Sequence: MPGFLVRILLLLLVLLLLGPTRGLRNATQRMFEIDYSRDSFLKDGQPFRYISGSIHYSRVPRFYWKDRLLKMKMAGLNAIQTYVPWNFHEPWPGQYQFSEDHDVEYFLRLAHELGLLVILRPGPYICAEWEMGGLPAWLLEKESILLRSSDPDYLAAVDKWLGVLLPKMKPLLYQNGGPVITVQVENEYGSYFACDFDYLRFLQKRFRHHLGDDVVLFTTDGAHKTFLKCGALQGLYTTVDFGTGSNITDAFLSQRKCEPKGPLINSEFYTGWLDHWGQPHSTIKTEAVASSLYDILARGASVNLYMFIGGTNFAYWNGANSPYAAQPTSYDYDAPLSEAGDLTEKYFALRNIIQKFEKVPEGPIPPSTPKFAYGKVTLEKLKTVGAALDILCPSGPIKSLYPLTFIQVKQHYGFVLYRTTLPQDCSNPAPLSSPLNGVHDRAYVAVDGIPQGVLERNNVITLNITGKAGATLDLLVENMGRVNYGAYINDFKGLVSNLTLSSNILTDWTIFPLDTEDAVRSHLGGWGHRDSGHHDEAWAHNSSNYTLPAFYMGNFSIPSGIPDLPQDTFIQFPGWTKGQVWINGFNLGRYWPARGPQLTLFVPQHILMTSAPNTITVLELEWAPCSSDDPELCAVTFVDRPVIGSSVTYDHPSKPVEKRLMPPPPQKNKDSWLDHV
• Conjugate: PE
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of GLB1 expression in transfected 293T cell line by GLB1 polyclonal antibody. Lane 1: GLB1 transfected lysate (76.1kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-GLB1 antibody

GLB1, a 60-76kD glycoprotein, is a lysosomal B-galactosidase that hydrolyzes the terminal B-galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome. In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0+/-3% of normal) of lysosomal B-galactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate. More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases. In lysosome, the mature B-galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex. An alternative splicing at the RNA level of GLB1 results a catalytically inactive B-galactosidase (also called elastin-binding protein) that plays an important role in vascular development.

Product Categories/Family for anti-GLB1 antibody

Antibodies; Enzymes; Glycosidase

NCBI and Uniprot Product Information

• NCBI GI #: 13960104
• NCBI GeneID: 2720
• NCBI Accession #: AAH07493.1
• NCBI GenBank Nucleotide #: NM_000404.1
• NCBI Official Full Name: Galactosidase, beta 1
• NCBI Official Synonym Full Names: galactosidase beta 1
• NCBI Official Symbol: GLB1
• NCBI Official Synonym Symbols: EBP; ELNR1; MPS4B
• NCBI Protein Information: beta-galactosidase
• Protein Family: Globulin

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]