SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• 동의어(들): GAA, LYAG, glucosidase alpha, acid
• UNSPSC 코드: 12352203
• NACRES: NA.46

속성

• 생물학적 소스: rabbit
• Quality Level: 100
• material: colorless
• 클론: 5L2L10, monoclonal
• 양식: liquid
• 종 반응성: rat, rat, human
• 농도: 0.8 mg/mL
• 기술: western blot: 1:500 - 1:2000
• 색상: colorless
• 동형: IgG
• 면역원 서열: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• 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

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

면역원

A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).

PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

애플리케이션

WB

안전성 정보

• Storage Class Code: 10 - Combustible liquids
• WGK: WGK 1