Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody

Descripción

HEXA Antibody is a Rabbit Polyclonal antibody against HEXA. This gene encodes the alpha 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. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).

Background

Hexosaminidase A (alpha polypeptide), also known as HEXA, corresponds to an enzyme encoded by the HEXA gene in humans. However, it's important to note that there is a chromosomal difference between humans and mice, with the HEXA gene located on chromosome 9 in humans and chromosome 15 in mice.The primary function of Hexosaminidase A, along with the cofactor GM2 activator protein, is to facilitate the degradation of GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines.The HEXA gene in humans spans approximately 26 kb and is composed of 14 exons and 13 introns. In conjunction with the HEXB gene, which encodes the beta-subunit, these genes collectively produce human beta-hexosaminidase, with HEXA responsible for the alpha-subunit. In mice, the equivalent genes are termed Hexa and Hexb.The subunits of beta-hexosaminidase, when they come together, form three distinct isozymes: beta-hexosaminidase A (alpha beta), beta-hexosaminidase B (beta beta), and beta-hexosaminidase S (alpha alpha). These isozymes possess the capability to break down various substrates that contain beta-linked N-acetylglucosamine and N-acetylgalactosamine residues.

Background

La hexosaminidasa A (polipéptido alfa), también conocida como HEXA, corresponde a una enzima codificada por el gen HEXA en humanos. Sin embargo, es importante tener en cuenta que existe una diferencia cromosómica entre humanos y ratones, con el gen HEXA ubicado en el cromosoma 9 en humanos y en el cromosoma 15 en ratones. La función principal de la hexosaminidasa A, junto con la proteína activadora cofactor GM2, es facilitar la degradación de los gangliósidos GM2 y otras moléculas que contienen N-acetil hexosaminas terminales. El gen HEXA en humanos abarca aproximadamente 26 kb y está compuesto por 14 exones y 13 intrones. Junto con el gen HEXB, que codifica la subunidad beta, estos genes producen colectivamente la beta-hexosaminidasa humana, siendo HEXA responsable de la subunidad alfa. En ratones, los genes equivalentes se denominan Hexa y Hexb. Las subunidades de la beta-hexosaminidasa, cuando se unen, forman tres isoenzimas distintas: beta-hexosaminidasa A (alfa beta), beta-hexosaminidasa B (beta beta) y beta- hexosaminidasa S (alfa alfa). Estas isoenzimas poseen la capacidad de descomponer varios sustratos que contienen residuos de N-acetilglucosamina y N-acetilgalactosamina con enlaces beta.

Características del producto

clonality

Polyclonal

reactivity

Human, Mouse, Rat

immunogen target

Beta-Hexosaminidase Subunit Alpha (HEXA)

host

Rabbit

conjugation

Unconjugated

form

Liquid

tested applications

purification

Purified by affinity chromatography.

buffer

PBS, pH 7.3, containing 0.05% Proclin-300, 50% glycerol.

size 1

20 µl

size 2

50 µl

size 3

100 µl

size 4

200 µl

storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.

or code

HEXA

dry ice

availability

Shipped within 5-10 working days.

alias

N-acetyl-beta-glucosaminidase subunit alpha,TSD

uniprot id

https://www.uniprot.org/uniprotkb/P06865

gene id

https://www.ncbi.nlm.nih.gov/gene/3073

NCBI accession

NP_000511.2

note

This product is for research use only.

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