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Mouse β-Hex A(β-hexosaminidase A) ELISA Kit
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935106861
info@markelab.com
Precio
Precio a consultar
proveedor
FineTestreference
EM1459Tested Applications
reactivity
mousestatus
RUOclonality
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
category
ELISA Kits
reactivity
mouse
test range
0.313-20ng/ml
assay type
Sandwich ELISA, Double Antibody
assay data
Quantitative
detection method
Colorimetric
sample type
Serum,Plasma,Tissue homogenates,Other biological fluids
size 1
96T
alias
β-Hex A|HEXA|TSD|beta-hexosaminidase subunit alpha|Beta-N-acetylhexosaminidase subunit alpha|hexosaminidase A|alpha polypeptide|Hexosaminidase subunit A|N-acetyl-beta-glucosaminidase subunit alpha
storage
2-8 °C for 6 months
or code
β-Hex A
uniprot id
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