Anti-HEXB monoclonal antibody (DCABH-3824) Knockout Validated

Rabbit anti-Human HEXB monoclonal antibody for WB, IHC-P

Specifications


Host Species
Rabbit
Antibody Isotype
IgG
Clone
FQS8089
Species Reactivity
Human
Immunogen
Synthetic peptide (the amino acid sequence is considered to be commercially sensitive)
Conjugate
Unconjugated

Applications


Application Notes
WB: 1/1000 - 1/10000; IHC-P: 1/25 - 1/50.
*Suggested working dilutions are given as a guide only. It is recommended that the user titrates the product for use in their own experiment using appropriate negative and positive controls.

Target


Alternative Names
HEXB; hexosaminidase B (beta polypeptide); beta-hexosaminidase subunit beta; HCC-7; hexosaminidase subunit B; beta-N-acetylhexosaminidase subunit beta
Entrez Gene ID
UniProt ID

Product Background


Gene summary
HEXB (Hexosaminidase Subunit Beta) is a Protein Coding gene. Diseases associated with HEXB include sandhoff disease, infantile, juvenile, and adult forms and gangliosidosis. Among its related pathways are Metabolism and Glycosphingolipid biosynthesis - globo series. GO annotations related to this gene include protein homodimerization activity and hydrolase activity, hydrolyzing O-glycosyl compounds. An important paralog of this gene is ENSG00000260729. Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Antigen Description
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. GM2-gangliosidosis 2 (GM2G2) [MIM:268800]: An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. Clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula. Note=The disease is caused by mutations affecting the gene represented in this entry. Beta-hexosaminidase subunit beta is an enzyme that in humans is encoded by the HEXB gene. 0The function about HEXB antigen include beta-N-acetylglucosaminidase activity; beta-N-acetylhexosaminidase activity; catalytic activity; cation binding; protein heterodimerization activity; protein heterodimerization activity; protein homodimerization activity; protein homodimerization activity.
Pathway
Amino sugar and nucleotide sugar metabolism, organism-specific biosystem; Amino sugar and nucleotide sugar 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 biosynthesis - globo series, organism-specific biosystem.

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References


Matsuoka, K; Tsuji, D; et al. Introduction of an N-Glycan Sequon Into HEXA Enhances Human beta-Hexosaminidase Cellular Uptake in a Model of Sandhoff Disease. MOLECULAR THERAPY 18:1519-1526(2010).
Buccoliero, R; Bodennec, J; et al. Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease. JOURNAL OF NEUROCHEMISTRY 90:80-88(2004).

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