WH0002548M1

Monoclonal Anti-GAA antibody produced in mouse

clone 3C6, purified immunoglobulin, buffered aqueous solution

• Sinónimos: Anti-LYAG, Anti-glucosidase, α acid (Pompe disease, glycogen storage disease type II)
• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• origen biológico: mouse
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: purified immunoglobulin
• tipo de anticuerpo: primary antibodies
• clon: 3C6, monoclonal
• Formulario: buffered aqueous solution
• reactividad de especies: human
• técnicas: indirect ELISA: suitable; western blot: 1-5 μg/mL
• isotipo: IgG1κ
• Nº de acceso GenBank: BC040431
• Nº de acceso UniProt: P10253
• Condiciones de envío: dry ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... GAA(2548)

Descripción

Descripción general

Lysosomal α-glucosidase (GAA), a glycoprotein and member of glycoside hydrolase family GH31, comprises a trefoil type-P domain, catalytic GH31 domain, distal, proximal, and an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome 17q25.3. It undergoes various proteolytical and N-glycan processing in the late endosomal/lysosomal compartment to become an active form.

Inmunógeno

GAA (AAH40431, 851 a.a. ~ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

Acciones bioquímicas o fisiológicas

Lysosomal α-glucosidase (GAA) uses mannose-6-phosphate receptors for its localization on the lysosomes. It mediates the hydrolysis of glycogen to glucose. Mutations in the GAA gene impair acid alpha-glucosidase enzyme activity. Deficiency of GAA leads to a rare lysosomal storage disease namely Pompe disease.

Forma física

Solution in phosphate buffered saline, pH 7.4

Información legal

GenBank is a registered trademark of United States Department of Health and Human Services

Cláusula de descargo de responsabilidad

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Información de seguridad

• Código de clase de almacenamiento: 10 - Combustible liquids
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable
• Equipo de protección personal: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)