MABT827-AF488

Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 488 Conjugate

clone 2C6 (MANDYS106), from mouse, ALEXA FLUOR^™ 488

• 동의어(들): Dystrophin
• UNSPSC 코드: 12352203
• eCl@ss: 32160702

속성

• 생물학적 소스: mouse
• Quality Level: 100
• 결합: ALEXA FLUOR^™ 488
• 항체 형태: purified immunoglobulin
• 항체 생산 유형: primary antibodies
• 클론: 2C6 (MANDYS106), monoclonal
• 종 반응성: human
• 기술: immunofluorescence: suitable
• 동형: IgG2aκ
• NCBI 수납 번호: NP_000100
• UniProt 수납 번호: P11532
• 배송 상태: ambient
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... DMD(1756)

설명

일반 설명

Dystrophin (UniProt P11532) is encoded by the DMD (also known as BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85) gene (Gene ID 1756) in human. Dystrophin is localized to the inner part of the muscle fiber cell membrane (sarcolemma), where it forms the dystrophin-associated glycoprotein complex (DGC) that links the extracellular matrix to the actin cytoskeleton. Dystrophin plays an important role in stabilizing the muscle fibre against the mechanical forces of muscle contraction by providing a shock-absorbing connection between the cytoskeleton and the extracellular matrix. Duchenne muscular dystrophy (DMD) is caused by gene mutations that disrupt the open reading frame (ORF) and prevent the full translation of dystrophin. ORF restoration by exon skipping using antisense oligonucleotides targeted to splicing elements are designed to transform the DMD phenotype to that of the milder disorder, Becker muscular dystrophy (BMD), typically caused by in-frame dystrophin deletions that allow the production of an internally deleted but partially functional dystrophin.

특이성

Detects dystrophin spliced isoforms 1-4, but not isoforms 5-10, or utrophin. Positive muscle membrane staining of tissue samples from healthy donors, reduced staining of Becker muscular dystrophy (BMD) biopsies, and no staining is seen with Duchenne muscular dystrophy (DMD) biopsy samples (Anthony, K., et al. (2011). Brain. 134(Pt 12):3547-3559).

면역원

TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.

애플리케이션

Research Category
Cell Structure

The unconjugated antibody (Cat. No. MABT827) and Alexa Fluor^™ 555 conjugate (Cat. No. MABT827-AF555) are also available for Immunohistochemistry, Immunofluorescence, and Western Blotting applications.

This mouse monoclonal Anti-Dystrophin Antibody, clone 2C6 (MANDYS106), Alexa Fluor^™ 488 Conjugate, Cat. No. MABT827-AF488 is validated for use in Immunofluorescence for the detection of Dystrophin.

품질

Evaluated by Immunofluorescence in human skeletal muscle tissue sections.

Immunofluorescence Analysis: A 1:25 dilution of this antibody detected dystrophin in human skeletal muscle tissue sections.

표적 설명

~427 kDa observed.

물리적 형태

Protein G purified.

Purified mouse IgG2a Alexa Fluor^™ 488 conjugate in buffer containing PBS, 1.5% BSA 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.

법적 정보

ALEXA FLUOR is a trademark of Life Technologies

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 2
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable