MABC974

Anti-Dysferlin/DYSF Antibody, clone 10H3.1

clone 10H3.1, from mouse

• 別名: Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, Dysferlin/DYSF
• UNSPSCコード: 12352203
• eCl@ss: 32160702
• NACRES: NA.43

特性

• 由来生物: mouse
• 品質水準: 100
• 抗体製品の状態: purified immunoglobulin
• 抗体製品タイプ: primary antibodies
• クローン: 10H3.1, monoclonal
• 化学種の反応性: human
• テクニック: immunohistochemistry: suitable (paraffin); western blot: suitable
• アイソタイプ: IgG2bκ
• NCBIアクセッション番号: NP_003485
• UniProtアクセッション番号: O75923
• 輸送温度: wet ice
• ターゲットの翻訳後修飾: unmodified
• 遺伝子情報: human ... DYSF(8291)

詳細

詳細

Dysferlin (UniProt O75923; also known as Dystrophy-associated fer-1-like 1 protein, Fer-1-like family member 1, Fer-1-like protein 1, Limb girdle muscular dystrophy 2B (autosomal recessive) is encoded by the DYSF (also known as FER1L1, LGMD2B, MMD1) gene (Gene ID 8291) in human. Dysferlin is a member of the ferlin family of calcium-dependent phospholipid-binding proteins involved in membrane vesicle fusion, membrane repair, T-tubule stability, and calcium homeostasis. Defects in dysferlin function/expression leads to muscular dystrophies (dysferlinopathies) that include Miyoshi myopathy (MM), limb-girdle muscular dystrophy type 2B (LGMD-2B), and distal myopathy with anterior tibial onset (DMAT).

特異性

Expected to react with spliced isoforms 1, 3, 4, 6, 9, 10, 12, 14, and 15. Reactivity toward isoforms 2, 5, 7, 8, 11, and 13 has not been tested.

免疫原

Epitope: N-terminal

GST-tagged recombinant protein corresponding to the N-terminal of human Dysferlin/DYSF.

アプリケーション

Research Category
アポトーシス及び癌

Research Sub Category
アポトーシス-追加

Immunohistochemistry Analysis: A 1:50-1,000 dilution from a representative lot detected Dysferlin/DYSF in human colon tissue and human skeletal muscle myocytes.

This Anti-Dysferlin/DYSF Antibody, clone 10H3.1 is validated for use in Western Blotting, Immunohistochemistry (Paraffin) for the detection of Dysferlin/DYSF.

品質

Evaluated by Western Blotting in human fetal skeletal muscle tissue lysate.

Western Blotting Analysis: 0.5 µg/mL of this antibody detected Dysferlin/DYSF in 10 µg of human fetal skeletal muscle tissue lysate.

ターゲットの説明

~237 kDa observed. Uncharacterized band(s) may appear in some lysates.

物理的形状

Protein G Purified

Format: Purified

Purified mouse monoclonal IgG2bκ antibody in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

保管および安定性

Stable for 1 year at 2-8°C from date of receipt.

その他情報

Concentration: Please refer to lot specific datasheet.

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.

安全性情報

• 保管分類コード: 12 - Non Combustible Liquids
• WGK: WGK 1
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable