MABC974

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

clone 10H3.1, from mouse

• 동의어(들): Dysferlin, Dystrophy-associated fer-1-like protein, Fer-1-like protein 1, Dysferlin/DYSF
• UNSPSC 코드: 12352203
• eCl@ss: 32160702
• NACRES: NA.43

속성

• 생물학적 소스: mouse
• Quality Level: 100
• 항체 형태: purified immunoglobulin
• 항체 생산 유형: primary antibodies
• 클론: 10H3.1, monoclonal
• 종 반응성: human
• 기술: immunohistochemistry: suitable (paraffin); western blot: suitable
• 동형: IgG2bκ
• NCBI 수납 번호: NP_003485
• UniProt 수납 번호: O75923
• 배송 상태: wet ice
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... DYSF(8291)

설명

일반 설명

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).

특이성

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.

면역원

Epitope: N-terminal

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

애플리케이션

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.

품질

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.

표적 설명

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

물리적 형태

Format: Purified

Protein G 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.

저장 및 안정성

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

기타 정보

Concentration: Please refer to lot specific datasheet.

면책조항

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.

안전성 정보

• Storage Class Code: 12 - Non Combustible Liquids
• WGK: WGK 1
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable