SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• Sinônimo(s): Anti-Glucosidase, α; acid, Anti-LYAG
• Código UNSPSC: 12352203
• NACRES: NA.41

Propriedades

• fonte biológica: rabbit
• Nível de qualidade: 100
• conjugado: unconjugated
• forma do anticorpo: affinity isolated antibody
• tipo de produto de anticorpo: primary antibodies
• clone: polyclonal
• Formulário: buffered aqueous solution
• peso molecular: 98 kDa
• reatividade de espécies: dog, guinea pig, human, rat, mouse, bovine
• concentração: 0.5 mg - 1 mg/mL
• técnica(s): immunohistochemistry: suitable; western blot: suitable
• nº de adesão UniProt: P10253
• Condições de expedição: wet ice
• temperatura de armazenamento: −20°C
• modificação pós-traducional do alvo: unmodified
• Informações sobre genes: human ... GAA(2548)

Descrição

Descrição geral

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.

Imunogênio

Synthetic peptide directed towards the N terminal region of human GAA

Aplicação

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

Ações bioquímicas/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.

Sequência

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.

Exoneração de responsabilidade

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.

Informações de segurança

• Código de classe de armazenamento: 10 - Combustible liquids
• Classe de risco de água (WGK): WGK 3
• Ponto de fulgor (°F): Not applicable
• Ponto de fulgor (°C): Not applicable