Beta-hexosaminidase subunit beta, Polyclonal Antibody

Beta hexosaminidase beta chain antibody; Beta hexosaminidase subunit beta antibody; Beta N acetylhexosaminidase antibody; Beta-hexosaminidase subunit beta chain A antibody; Beta-N-acetylhexosaminidase subunit beta antibody; Cervical cancer proto oncogene 7 protein antibody; Cervical cancer proto-oncogene 7 protein antibody; ENC 1AS antibody; HCC 7 antibody; HCC-7 antibody; HCC7 antibody; HEX B antibody; Hexb antibody; HEXB_HUMAN antibody; Hexosaminidase B (beta polypeptide) antibody; Hexosaminidase B antibody; Hexosaminidase subunit B antibody; HexosaminidaseB antibody; N acetyl beta glucosaminidase antibody; N-acetyl-beta-glucosaminidase subunit beta antibody; hexosaminidase B (beta polypeptide)

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-HEXB antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: Rabbit IgG polyclonal antibody for Beta-hexosaminidase subunit beta(HEXB) detection. Tested with WB, IHC-P in Human.
Background: 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.

Western Blot (WB), Immunohistochemistry (IHC-P)

Western Blot: Concentration: 0.1-0.5mug/ml; Tested Species: Human
Immunohistochemistry (Paraffin-embedded Section): Concentration: 0.5-1mug/ml; Tested Species: Human

Anti- HEXB antibody, MBS177499, Western blotting
All lanes: Anti HEXB (MBS177499) at 0.5ug/ml
WB: Recombinant Human HEXB Protein 0.5ng
Predicted bind size: 38KD
Observed bind size: 38KD

Anti- HEXB antibody, MBS177499, Western blotting
All lanes: Anti HEXB (MBS177499) at 0.5ug/ml
Lane 1: HELA Whole Cell Lysate at 40ug
Lane 2: HEPG2 Whole Cell Lysate at 40ug
Predicted bind size: 63KD
Observed bind size: 63KD

Anti- HEXB antibody, MBS177499,IHC(P)
IHC(P): Human Lung Cancer Tissue

63,111 Da

hexosaminidase B (beta polypeptide)

beta-hexosaminidase subunit beta; HCC-7; hexosaminidase subunit B; epididymis luminal protein 248; beta-N-acetylhexosaminidase subunit beta; cervical cancer proto-oncogene 7 protein; N-acetyl-beta-glucosaminidase subunit beta

Beta-hexosaminidase subunit beta

Hexosaminidase subunit B; HCC-7  [Similar Products]

HEXB_HUMAN

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. [provided by RefSeq, May 2014]

HEXB: Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. Defects in HEXB are the cause of GM2-gangliosidosis type 2 (GM2G2); also known as Sandhoff disease. GM2- gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G2 is 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. Belongs to the glycosyl hydrolase 20 family.

Protein type: EC 3.2.1.52; Hydrolase; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosaminoglycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Glycan Metabolism - other glycan degradation; Carbohydrate Metabolism - amino sugar and nucleotide sugar

Chromosomal Location of Human Ortholog: 5q13

Cellular Component: lysosomal lumen; membrane; acrosome

Molecular Function: protein homodimerization activity; protein heterodimerization activity; beta-N-acetylhexosaminidase activity

Biological Process: oogenesis; male courtship behavior; myelination; keratan sulfate metabolic process; glycosaminoglycan metabolic process; ganglioside catabolic process; locomotory behavior; pathogenesis; hyaluronan catabolic process; regulation of cell shape; oligosaccharide catabolic process; sequestering of lipid; sensory perception of sound; chondroitin sulfate catabolic process; penetration of zona pellucida; keratan sulfate catabolic process; neuromuscular process controlling balance; skeletal development; sphingolipid metabolic process; phospholipid biosynthetic process; cellular calcium ion homeostasis; chondroitin sulfate metabolic process; cellular protein metabolic process; lysosome organization and biogenesis; carbohydrate metabolic process; positive regulation of transcription from RNA polymerase II promoter; glycosphingolipid metabolic process; hyaluronan metabolic process; astrocyte cell migration

Disease: Sandhoff Disease

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