MilliporeSigma™ Dystrophin, Mouse, Alexa Fluor 488, Clone: 2C6 (MANDYS106) ,
Manufacturer: MilliporeSigma™
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | MABT827AF48-Each-of-1 | In Stock | ₹ 38,958.86 |
MABT827AF48 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 38,958.86
GST (18%): ₹ 7,012.595
Total Price: ₹ 45,971.455
Antigen
Dystrophin
Classification
Monoclonal
Concentration
Please refer to lot specific datasheet.
Formulation
Purified mouse IgG2a Alexa Fluor™ 488 conjugate in buffer containing PBS, 1.5% BSA with 0.05% Sodium Azide.
Gene Accession No.
P11532
Immunogen
TrpE-tagged recombinant protein corresponding to the Exon 43-coded pectrin-like repeat 16 region of human Dystrophin.
Quantity
100 μL
Research Discipline
Cell Structure
Gene ID (Entrez)
NP_000100
Target Species
Human
Isotype
IgG2a κ
Applications
Immunofluorescence
Clone
2C6 (MANDYS106)
Conjugate
Alexa Fluor 488
Gene
DMD, BMD, CMD3B, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, MRX85
Host Species
Mouse
Purification Method
Protein G Purified
Regulatory Status
RUO
Primary or Secondary
Primary
Test Specificity
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).
Content And Storage
Stable for 1 year at 2°-8°C from date of receipt.
Related Products
Description
- 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.