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由来生物
rabbit
品質水準
結合体
unconjugated
抗体製品の状態
affinity isolated antibody
抗体製品タイプ
primary antibodies
クローン
polyclonal
形状
buffered aqueous solution
分子量
antigen 237 kDa
化学種の反応性
mouse, human
濃度
~1 mg/mL
テクニック
ELISA: 1:10000
western blot: 1:500-1:1000
NCBIアクセッション番号
UniProtアクセッション番号
輸送温度
wet ice
保管温度
−20°C
ターゲットの翻訳後修飾
unmodified
遺伝子情報
human ... DYSF(8291)
詳細
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
アプリケーション
Anti-Dysferlin, antibody produced in rabbit has been used in western blotting.
生物化学的／生理学的作用
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).
特徴および利点
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.
物理的形状
ウサギIgGのPBS溶液（Mg2+およびCa2+を含まず）、pH 7.4、150 mM NaCl、0.02％ アジ化ナトリウム、50％ グリセロール
免責事項
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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保管分類コード
10 - Combustible liquids
WGK
nwg
引火点(°F)
Not applicable
引火点(℃)
Not applicable
試験成績書（COA）
製品のロット番号・バッチ番号を入力して、試験成績書（COA） を検索できます。ロット番号・バッチ番号は、製品ラベルに「Lot」または「Batch」に続いて記載されています。
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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