₩824,891
예상 입고일2025년 5월 29일세부사항
추천 제품
생물학적 소스
rabbit
Quality Level
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
배송 상태
wet ice
저장 온도
−20°C
타겟 번역 후 변형
unmodified
유전자 정보
human ... GAA(2548)
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
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.
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Storage Class Code
10 - Combustible liquids
WGK
WGK 3
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
가장 최신 버전 중 하나를 선택하세요:
Cinzia Bragato et al.
Biochimica et biophysica acta. Molecular basis of disease, 1866(5), 165662-165662 (2020-01-10)
Pompe disease (PD) is an autosomal recessive muscular disorder caused by deficiency of the glycogen hydrolytic enzyme acid α-glucosidase (GAA). The enzyme replacement therapy, currently the only available therapy for PD patients, is efficacious in improving cardiomyopathy in the infantile
Hossein Moravej et al.
Iranian journal of medical sciences, 43(2), 218-222 (2018-05-12)
Pompe disease (PD), also known as "glycogen storage disease type II (OMIM # 232300)" is a rare autosomal recessive disorder characterized by progressive glycogen accumulation in cellular lysosomes. It ultimately leads to cellular damage. Infantile-onset Pompe disease (IOPD) is the
Xiaodong Jia et al.
Aging, 12(5), 4268-4282 (2020-03-04)
Clinical manifestations of the late-onset adult Pompe disease (glycogen storage disease type II) are heterogeneous. To identify genetic defects of a special patient population with cerebrovascular involvement as the main symptom, we performed whole-genome sequencing (WGS) analysis on a consanguineous
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
Véronique Roig-Zamboni et al.
Nature communications, 8(1), 1111-1111 (2017-10-25)
Pompe disease, a rare lysosomal storage disease caused by deficiency of the lysosomal acid α-glucosidase (GAA), is characterized by glycogen accumulation, triggering severe secondary cellular damage and resulting in progressive motor handicap and premature death. Numerous disease-causing mutations in the
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