SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• Sinónimos: Anti-Glucosidase, α; acid, Anti-LYAG
• Código UNSPSC: 12352203
• NACRES: NA.41

Propiedades

• origen biológico: rabbit
• Nivel de calidad: 100
• conjugado: unconjugated
• forma del anticuerpo: affinity isolated antibody
• tipo de anticuerpo: primary antibodies
• clon: polyclonal
• Formulario: buffered aqueous solution
• mol peso: 98 kDa
• reactividad de especies: dog, guinea pig, human, rat, mouse, bovine
• concentración: 0.5 mg - 1 mg/mL
• técnicas: immunohistochemistry: suitable; western blot: suitable
• Nº de acceso UniProt: P10253
• Condiciones de envío: wet ice
• temp. de almacenamiento: −20°C
• modificación del objetivo postraduccional: unmodified
• Información sobre el gen: human ... GAA(2548)

Descripción

Descripción general

Lysosomal alpha-glucosidase (GAA) is a glycoprotein that belongs to the glycoside hydrolase family GH31. It comprises a catalytic GH31 (β/α)8 barrel domain, trefoil type-P domain, an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome location 17q25.3.

Inmunógeno

Synthetic peptide directed towards the N terminal region of human GAA

Aplicación

Anti-GAA antibody produced in rabbit has been used western blotting (1:100)

Acciones bioquímicas o fisiológicas

GAA is acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

Secuencia

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Forma física

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

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
• Clase de riesgo para el agua (WGK): WGK 3
• Punto de inflamabilidad (°F): Not applicable
• Punto de inflamabilidad (°C): Not applicable