Anti-HEXB Antibody Picoband® Fluoro488 Conjugated

Beta-hexosaminidase subunit beta (HEXB) is an enzyme that in humans is encoded by the HEXB gene. It is mapped to 5q13.3. HEXB 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 subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II) . It has been found that HEXB is a peptidoglycan hydrolase and it is involved in restricting mycobacteria growth even before the onset of adaptive immunity.

Beta-hexosaminidase subunit beta;3.2.1.52; Beta-N-acetylhexosaminidase subunit beta; Hexosaminidase subunit B; Cervical cancer proto-oncogene 7 protein; HCC-7; N-acetyl-beta-glucosaminidase subunit beta; Beta-hexosaminidase subunit beta chain B; Beta-hexosaminidase subunit beta chain A; HEXB; HCC7

E.coli-derived human HEXB recombinant protein (Position: K381-M556) . Human HEXB shares 75% and 73% amino acid (aa) sequences identity with mouse and rat HEXB, respectively.

Detected in brain, in particular in amygdala and hippocampus, while expression in caudate nucleus, corpus callosum, substantia nigra, subthalamic nucleus and thalamus is very low or not detectable. Detected at very low levels in muscle and pancreas. .

Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.

1. O'Dowd BF, Quan F, Willard HF, Lamhonwah AM, Korneluk RG, Lowden JA, Gravel RA, Mahuran DJ (Apr 1985) . Isolation of cDNA clones coding for the beta subunit of human beta-hexosaminidase. Proc Natl Acad Sci U S A 82 (4) : 1184–8. 2. Korneluk RG, Mahuran DJ, Neote K, Klavins MH, O'Dowd BF, Tropak M, Willard HF, Anderson MJ, Lowden JA, Gravel RA (Jul 1986) . Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease. J Biol Chem 261 (18) : 8407–13. 3. Entrez Gene: HEXB hexosaminidase B (beta polypeptide)