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Anti-Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)(DMD/3241), CF488A conjugate

Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2. 4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0. 002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission. Primary antibodies are available purified, or with a selection of fluorescent CF® Dyes and other labels. CF® Dyes offer exceptional brightness and photostability. Note: Conjugates of blue fluorescent dyes like CF®405S and CF®405M are not recommended for detecting low abundance targets, because blue dyes have lower fluorescence and can give higher non-specific background than other dye colors.

Product Specifications

Synonyms

BMD; CMD3B; Duchenne muscular dystrophy (DMD); Dystrophin; Muscular dystrophy Duchenne and Becker types

UNSPSC

41116161

UNSPSC Description

Primary and secondary antibodies for multiple methodology immunostaining detection application

Gene Name

DMD

Gene ID

1756

NCBI Gene ID

495912

UniProt

P11532

Cellular Locus

Plasma membrane

Host

Mouse

Species Reactivity

Human

Immunogen

A recombinant fragment (around aa 114-263) of human DMD protein (exact sequence is proprietary)

Target Antigen

Dystrophin

Clonality

Monoclonal

Isotype

IgG1 κ

Clone

DMD/3241

Conjugation

CF488A

Source

Animal

Applications

IHC, FFPE (verified)

Validated Applications

IHC,FFPE

Positive Control

Human skeletal muscle and heart muscle tissues (IHC).

Concentration

0.1 mg/mL

Buffer

PBS, 0.1% BSA, 0.05% azide

Molecular Weight

427 kDa

Shipping Conditions

Room temperature

Storage Conditions

4°C; Protect from light; Stable at room temperature or 37°C (98°F) for 7 days.

Shelf Life

2 years

Product Datasheet

https://cdn.gentaur.com/products/37/7649485/datasheet/bnc883241-100.pdf

Product MSDS

https://cdn.gentaur.com/products/37/7649485/msds/bnc883241-100.pdf

CAS Number

9007-83-4

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