Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) (DMD/3242), 0.2mg/mL
Selected Variant
BNUB3242-500
Size: 1x 500 µL
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
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/3242
Type
Primary
Source
Animal
Origin
Animal
Applications
IHC, FFPE (verified)
Positive Control
Human skeletal muscle and heart muscle tissues (IHC).
Stability
2 years
Concentration
0.2 mg/mL
Buffer
PBS, 0.05% 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.
Shelf Life
Guaranteed for at least 24 months from date of receipt when stored as recommended
Fragment
IgG2b, kappa
Classification
PBS, 0.05% BSA, 0.05% sodium azide (CAS 26628-22-8)
Conjugate
Purified, with BSA
Price
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