Dystrophin antibody (AA 114-263) (CF®647)
Quick Overview for Dystrophin antibody (AA 114-263) (CF®647) (ABIN7653316)
Target
See all Dystrophin (DMD) AntibodiesReactivity
Host
Clonality
Conjugate
Application
Clone
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Binding Specificity
 - AA 114-263
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Purpose
 - Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy)(DMD/3243), CF647 conjugate
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Characteristics
 - 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.
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Immunogen
 - A recombinant fragment (around aa 114-263) of human DMD protein
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Isotype
 - IgG1, kappa
 
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Application Notes
 - Optimal working dilution should be determined by the investigator.
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Comment
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Positive Control: Human skeletal muscle and heart muscle tissues (IHC).
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Restrictions
 - For Research Use only
 
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Format
 - Liquid
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Concentration
 - 0.1 mg/mL
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Buffer
 - PBS, 0.1 % BSA, 0.05 % azide
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Preservative
 - Sodium azide
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Precaution of Use
 - This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Handling Advice
 - Protect from light
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Storage
 - 4 °C
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Storage Comment
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Stable at room temperature or 37°C for 7 days.
Protect from light
Store at 2 to 8°C. Protect fluorescent conjugates from light
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Expiry Date
 - 24 months
 
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- Dystrophin (DMD)
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Alternative Name
 - Dystrophin
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Background
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Synonyms: BMD, CMD3B, Duchenne muscular dystrophy (DMD), Dystrophin, Muscular dystrophy Duchenne and Becker types
Gene Symbol: DMD
Tissue Expression: Muscle
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Molecular Weight
 - 427 kDa
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Gene ID
 - 1756
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UniProt
 - P11532
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Pathways
 - Skeletal Muscle Fiber Development
 
Target
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