SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• Synonim(y): Anti-Glucosidase, α; acid, Anti-LYAG
• Kod UNSPSC: 12352203
• NACRES: NA.41

Właściwości

• pochodzenie biologiczne: rabbit
• Poziom jakości: 100
• białko sprzężone: unconjugated
• forma przeciwciała: affinity isolated antibody
• rodzaj przeciwciała: primary antibodies
• klon: polyclonal
• Formularz: buffered aqueous solution
• masa cząsteczkowa: 98 kDa
• reaktywność gatunkowa: dog, guinea pig, human, rat, mouse, bovine
• stężenie: 0.5 mg - 1 mg/mL
• metody: immunohistochemistry: suitable; western blot: suitable
• numer dostępu UniProt: P10253
• Warunki transportu: wet ice
• temp. przechowywania: −20°C
• docelowa modyfikacja potranslacyjna: unmodified
• informacje o genach: human ... GAA(2548)

Opis

Opis ogólny

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.

Immunogen

Synthetic peptide directed towards the N terminal region of human GAA

Zastosowanie

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

Działania biochem./fizjol.

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.

Sekwencja

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Postać fizyczna

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

Oświadczenie o zrzeczeniu się odpowiedzialności

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.

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Informacja o środkach bezpieczeństwa

• Kod klasy składowania: 10 - Combustible liquids
• Klasa zagrożenia wodnego (WGK): WGK 3
• Temperatura zapłonu (°F): Not applicable
• Temperatura zapłonu (°C): Not applicable