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NeoBiotechnologies
Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Monoclonal Antibody (DMD/3676)
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Product Details
1756-MSM6-P1
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG2b, kappa
Class
Monoclonal
Type
Antibody
Clone
DMD/3676
Immunogen
A recombinant fragment (around aa 114-263) of human DMD protein.
Conjugate
Form
Liquid
Concentration
200 µg/mL
Purification
Protein A/G
Storage buffer
PBS with 0.05% BSA
Contains
0.05% sodium azide
Storage conditions
4°C
Shipping conditions
Ambient (domestic); Wet ice (international)
Product Specific Information
Antibody is stable for 24 months.
Positive Control: Human skeletal muscle and heart muscle tissues (IHC). Cellular Localization: Cell surface. Cytoplasm.
Specificity Comments: 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.
Target Information
Dystrophin is the 427kDa protein product of the DMB/BMD gene located on the X chromosome at position Xp21. Western blotting and immunohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies. Dystrophin abnormalities are thought to occur in 100% of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65% of cases.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Duchenne muscular dystrophy (DMD); Dystrophin; GS1-19O24.1; Muscular dystrophy Duchenne and Becker types; OTTHUMP00000215592
Gene Aliases: BMD; CMD3B; DMD; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; MRX85
UniProt ID: (Human) P11532
Entrez Gene ID: (Human) 1756
regulation of heart rate
cytoskeleton organization
muscle organ development
regulation of release of sequestered calcium ion into cytosol by sarcoplasmic reticulum
regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ion
regulation of skeletal muscle contraction by regulation of release of sequestered calcium ion
regulation of skeletal muscle contraction
muscle attachment
muscle filament sliding
negative regulation of peptidyl-serine phosphorylation
cellular protein localization
response to muscle stretch
peptide biosynthetic process
cellular protein complex assembly
muscle cell cellular homeostasis
muscle fiber development
cardiac muscle contraction
regulation of ryanodine-sensitive calcium-release channel activity
cardiac muscle cell action potential
regulation of voltage-gated calcium channel activity
negative regulation of peptidyl-cysteine S-nitrosylation
positive regulation of sodium ion transmembrane transporter activity
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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