SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• 동의어(들): Anti-Glucosidase, α; acid, Anti-LYAG
• UNSPSC 코드: 12352203
• NACRES: NA.41

속성

• 생물학적 소스: rabbit
• Quality Level: 100
• 결합: unconjugated
• 항체 형태: affinity isolated antibody
• 항체 생산 유형: primary antibodies
• 클론: polyclonal
• 양식: buffered aqueous solution
• 분자량: 98 kDa
• 종 반응성: dog, guinea pig, human, rat, mouse, bovine
• 농도: 0.5 mg - 1 mg/mL
• 기술: immunohistochemistry: suitable; western blot: suitable
• UniProt 수납 번호: P10253
• 배송 상태: wet ice
• 저장 온도: −20°C
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... GAA(2548)

설명

일반 설명

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.

면역원

Synthetic peptide directed towards the N terminal region of human GAA

애플리케이션

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

생화학적/생리학적 작용

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.

서열

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

물리적 형태

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

면책조항

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.

안전성 정보

• Storage Class Code: 10 - Combustible liquids
• WGK: WGK 3
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable