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Anti-HEXA Antibody

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产品名称: Anti-HEXA Antibody
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简单介绍

Anti-HEXA Antibody


Anti-HEXA Antibody  的详细介绍
Name: Anti-HEXA Antibody
See all HEXA primary antibodies
Description: Rabbit polyclonal antibody to HEXA
Specificity: HEXA pAb detects endogenous levels of HEXA protein.
Applications: WB, IHC
Reactivity: Human, Mouse, Rat
Immunogen: Recombinant full length Human HEXA.
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Molecular Weight: ~60 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Product Form: 1mg/ml in PBS?with?0.1%?Sodium?Azide,?50%?Glycerol.
Function: Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Involvement in Disease: GM2-gangliosidosis 1: An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. It is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. It exists in several forms: infantile (most common and most severe), juvenile and ***** (late-onset).
Sequence Similarities: Belongs to the glycosyl hydrolase 20 family.
Post-Translational Modification: N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).
Cellular Location: Lysosome.
Database Links:
  • Entrez Gene: 3073?Human
  • Entrez Gene: 15211?Mouse
  • Entrez Gene: 300757?Rat
  • Omim: 606869?Human
  • SwissProt: P06865?Human
  • SwissProt: P29416?Mouse
  • SwissProt: Q641X3?Rat
  • Unigene: 604479?Human
  • Unigene: 709495?Human
  • Unigene: 2284?Mouse
  • Unigene: 92939?Rat
    		•
    Synonyms:
  • Beta hexosaminidase alpha chain precursor Antibody
  • Beta hexosaminidase subunit alpha Antibody
  • Beta N acetylhexosaminidase Antibody
  • Beta N acetylhexosaminidase subunit alpha Antibody
  • Beta-hexosaminidase A Antibody
  • Beta-hexosaminidase subunit alpha Antibody
  • Beta-N-acetylhexosaminidase subunit alpha Antibody
  • HEXA Antibody
  • HEXA_HUMAN Antibody
  • Hexosaminidase A Antibody
  • Hexosaminidase A (alpha polypeptide) Antibody
  • Hexosaminidase A alpha polypeptide Antibody
  • Hexosaminidase subunit A Antibody
  • MGC99608 Antibody
  • N acetyl beta glucosaminidase Antibody
  • N acetyl beta glucosaminidase subunit alpha Antibody
  • N-acetyl-beta-glucosaminidase subunit alpha Antibody
  • TSD Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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