생물학적 소스
rabbit
Quality Level
결합
unconjugated
항체 형태
IgG fraction of antiserum
항체 생산 유형
primary antibodies
클론
polyclonal
형태
buffered aqueous solution
분자량
43 kDa
종 반응성
human, rabbit, guinea pig, mouse, horse, dog, bovine, rat
농도
0.5 mg - 1 mg/mL
기술
immunohistochemistry: suitable
western blot: suitable
NCBI 수납 번호
UniProt 수납 번호
배송 상태
wet ice
저장 온도
−20°C
유전자 정보
human ... GMPPB(29925)
면역원
Synthetic peptide directed towards the C terminal region of human GMPPB
애플리케이션
Anti-GMPPB antibody produced in rabbit is suitable for western blotting at a concentration of 5.0μg/ml and for immunohistochemistry of paraffin-embedded tissue sections at a concentration of 4-8μg/ml.
생화학적/생리학적 작용
GDP-mannose pyrophosphorylase B (GMPPB) catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose during the synthesis of N-linked oligosaccharides. Mutations in GMPPB gene results in hypoglycosylation of α-dystroglycan characteristic of congenital and limb-girdle muscular dystrophies.
서열
Synthetic peptide located within the following region: RCRVGQWVRMENVTVLGEDVIVNDELYLNGASVLPHKSIGESVPEPRIIM
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
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
Keren J Carss et al.
American journal of human genetics, 93(1), 29-41 (2013-06-19)
Congenital muscular dystrophies with hypoglycosylation of α-dystroglycan (α-DG) are a heterogeneous group of disorders often associated with brain and eye defects in addition to muscular dystrophy. Causative variants in 14 genes thought to be involved in the glycosylation of α-DG
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