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Key Documents

SAB2100872

Sigma-Aldrich

Anti-GAA antibody produced in rabbit

affinity isolated antibody

Sinonimo/i:

Anti-Glucosidase, α; acid, Anti-LYAG

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About This Item

Codice UNSPSC:
12352203
NACRES:
NA.41

Origine biologica

rabbit

Livello qualitativo

Coniugato

unconjugated

Forma dell’anticorpo

affinity isolated antibody

Tipo di anticorpo

primary antibodies

Clone

polyclonal

Forma fisica

buffered aqueous solution

PM

98 kDa

Reattività contro le specie

dog, guinea pig, human, rat, mouse, bovine

Concentrazione

0.5 mg - 1 mg/mL

tecniche

immunohistochemistry: suitable
western blot: suitable

N° accesso UniProt

Condizioni di spedizione

wet ice

Temperatura di conservazione

−20°C

modifica post-traduzionali bersaglio

unmodified

Informazioni sul gene

human ... GAA(2548)

Descrizione generale

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.

Immunogeno

Synthetic peptide directed towards the N terminal region of human GAA

Applicazioni

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

Azioni biochim/fisiol

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.

Sequenza

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

Stato fisico

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

Esclusione di responsabilità

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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Codice della classe di stoccaggio

10 - Combustible liquids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable


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Francesco Chemello et al.
Cell reports, 26(13), 3784-3797 (2019-03-28)
Skeletal muscle is composed of different myofiber types that preferentially use glucose or lipids for ATP production. How fuel preference is regulated in these post-mitotic cells is largely unknown, making this issue a key question in the fields of muscle

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