Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) (DMD/3244), CF594 conjugate, 0.1mg/mL

About this product

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.

Specifications

Catalog number

BNC943244-100

Size / count

1x 100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9007-83-4

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

Swiss Prot

P11532

Host

Mouse

Species Reactivity

Human

Reactivity

Dystrophin

Immunogen

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

Target Antigen

Dystrophin

Clonality

Monoclonal

Clone

DMD/3244

Type

Primary

Source

Animal

Origin

Animal

Applications

IHC, FFPE (verified)

Positive Control

Human skeletal muscle and heart muscle tissues (IHC).

Stability

2 years

Concentration

0.1 mg/mL

Buffer

PBS, 0.1% BSA, 0.05% azide

Molecular Weight

427 kDa

Shipping Conditions

RT (ND)

Storage Conditions

Stable at room temperature or 37°C (98°F) for 7 days.

Notes

Protect from light

Shelf Life

Guaranteed for at least 24 months from date of receipt when stored as recommended

Fragment

IgG1, kappa

Classification

0.05% sodium azide (CAS 26628-22-8)

Conjugate

CF594