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生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
形狀
buffered aqueous solution
分子量
antigen 237 kDa
物種活性
mouse, human
濃度
~1 mg/mL
技術
ELISA: 1:10000
western blot: 1:500-1:1000
NCBI登錄號
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... DYSF(8291)
一般說明
Dysferlin (DYSF) is a large transmembrane protein that belongs to the ferlin family of Ca2+-dependent phospholipid-binding proteins. It is expressed in skeletal and cardiac muscles. DYSF gene is mapped to human chromosome 2p13. Anti-Dysferlin antibody detects endogenous levels of total dysferlin protein.
免疫原
The antiserum was produced against synthesized peptide derived from human Dysferlin.
Immunogen Range: 1981-2030
Immunogen Range: 1981-2030
應用
Anti-Dysferlin, antibody produced in rabbit has been used in western blotting.
生化／生理作用
Dysferlin (DYSF) plays a key role in membrane repair, vesicle trafficking, and Ttubule structure. Lack of DYSF protein expression in skeletal muscles leads to dysferlinopathy. Mutations in the DYSF gene are associated with Miyoshi myopathy, limb-girdle muscular dystrophy type 2B (LGMD2B), and distal myopathy with anterior tibial onset (DMAT).
特點和優勢
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
外觀
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
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）
nwg
閃點（°F）
Not applicable
閃點（°C）
Not applicable
Jin Tang et al.
Neuropathology : official journal of the Japanese Society of Neuropathology (2018-05-26)
Dysferlinopathy, a progressive muscular dystrophy, results from mutations in the Dysferlin gene (DYSF, MIM*603009). Traditional diagnosis relies on the reduction or absence of dysferlin. However, altered dysferlin has been observed in other myopathies, leading to a precise diagnosis through molecular
Functional recovery of a novel knock-in mouse model of dysferlinopathy by readthrough of nonsense mutation
Seo K, et al.
Molecular therapy. Methods & clinical development (2021)
Kyowon Seo et al.
Molecular therapy. Methods & clinical development, 21, 702-709 (2021-06-19)
Biallelic mutations in the dysferlin gene cause limb-girdle muscular dystrophy 2B or Miyoshi distal myopathy. We found that nonsense mutations are the most common mutation type among Korean patients with dysferlinopathy; more than half of the patients have at least
Janice A Dominov et al.
Annals of clinical and translational neurology, 6(4), 642-654 (2019-04-26)
Dysferlin is a large transmembrane protein that functions in critical processes of membrane repair and vesicle fusion. Dysferlin-deficiency due to mutations in the dysferlin gene leads to muscular dystrophy (Miyoshi myopathy (MM), limb girdle muscular dystrophy type 2B (LGMD2B), distal
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