SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• Synonyme(s) : GAA, LYAG, glucosidase alpha, acid
• Code UNSPSC : 12352203
• Nomenclature NACRES : NA.46

Propriétés

• Source biologique: rabbit
• Niveau de qualité: 100
• Matériaux: colorless
• Clone: 5L2L10, monoclonal
• Forme: liquid
• Espèces réactives: rat, rat, human
• Concentration: 0.8 mg/mL
• Technique(s): western blot: 1:500 - 1:2000
• Couleur: colorless
• Isotype: IgG
• Séquence immunogène: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• Numéro d'accès UniProt: P10253
• Conditions d'expédition: wet ice
• Température de stockage: −20°C
• Modification post-traductionnelle de la cible: unmodified
• Informations sur le gène: human ... GAA(2548)

Description

Description générale

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe′s disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

Immunogène

A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Application

WB

Informations sur la sécurité

• Code de la classe de stockage: 10 - Combustible liquids
• Classe de danger pour l'eau (WGK): WGK 1