生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
IgG fraction of antiserum
抗體產品種類
primary antibodies
無性繁殖
polyclonal
形狀
buffered aqueous solution
分子量
43 kDa
物種活性
human, rabbit, guinea pig, mouse, horse, dog, bovine, rat
濃度
0.5 mg - 1 mg/mL
技術
immunohistochemistry: suitable
western blot: suitable
NCBI登錄號
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
基因資訊
human ... GMPPB(29925)
免疫原
Synthetic peptide directed towards the C terminal region of human GMPPB
應用
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.
生化／生理作用
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.
序列
Synthetic peptide located within the following region: RCRVGQWVRMENVTVLGEDVIVNDELYLNGASVLPHKSIGESVPEPRIIM
外觀
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
免責聲明
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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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
分析證明 (COA)
輸入產品批次/批號來搜索 分析證明 (COA)。在產品’s標籤上找到批次和批號，寫有 ‘Lot’或‘Batch’.。
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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