MABC974

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

clone 10H3.1, from mouse

• 同義詞: Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, Dysferlin/DYSF
• 分類程式碼代碼: 12352203
• eCl@ss: 32160702

屬性

• 生物源: mouse
• 品質等級: 100
• 抗體表格: purified immunoglobulin
• 抗體產品種類: primary antibodies
• 無性繁殖: 10H3.1, monoclonal
• 物種活性: human
• 技術: immunohistochemistry: suitable (paraffin); western blot: suitable
• 同型: IgG2bκ
• NCBI登錄號: NP_003485
• UniProt登錄號: O75923
• 運輸包裝: wet ice
• 目標翻譯後修改: unmodified
• 基因資訊: human ... DYSF(8291)

描述

一般說明

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.

應用

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

Research Category
Apoptosis & Cancer

Research Sub Category
Apoptosis - Additional

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.

標靶描述

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

外觀

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.

儲存和穩定性

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

其他說明

Concentration: Please refer to lot specific datasheet.

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.

安全資訊

• 儲存類別代碼: 12 - Non Combustible Liquids
• 水污染物質分類（WGK）: WGK 1
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable