BS8151

antibody from Bioworld Technology, Inc
Targeting: AGA ASRG
Provider product page for BS8151
Western blot
Immunocytochemistry
Immunohistochemistry

Antibody data

Product number
BS8151 - Provider product page
Provider
Bioworld Technology, Inc
Product name
AGA polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human AGA.
Description
AGA (aspartylglucosaminidase) is a 346 amino acid precursor protein that belongs to the Ntn-hydrolase family and is cleaved to produce an Ï« chain and a ÉÇ chain. Localized to the lysosome, AGA functions as a heterotetramer composed of two Ï« and two ÉÇ chains that work together to cleave the GlcNAc-Asn bond that joins oligosaccharides to target glycoproteins. Defects in the gene encoding AGA are the cause of aspartylglucosaminuria (AGU), a lysosomal storage disease that is characterized by severe mental retardation and mild connective tissue abnormalities. The gene encoding AGA maps to human chromosome 4, which encodes nearly 6% of the human genome and has the largest gene deserts (regions of the genome with no protein encoding genes) of all of the human chromosomes.
Reactivity
Human, Mouse
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
AGA protein structure - BS8151 shown in red.