Orthogonal Strategies: Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining in human skeletal muscle and tonsil tissues . Corresponding DMD RNA-seq data are presented for the same tissues.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human heart muscle shows strong membranous positivity in myocytes.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human skeletal muscle shows strong membranous positivity in myocytes.
Immunohistochemistry-Paraffin: Dystrophin Antibody [NBP1-89953] - Staining of human tonsil shows no positivity as expected.
This antibody was developed against Recombinant Protein corresponding to amino acids: KQNDVHRAFKRELKTKEPVIMSTLETVRIFLTEQPLEGLEKLYQEPRELPPEERAQNVTRLLRKQAEEVNTEWEKLNLHSADWQRKIDETLERLQELQEATDELDLKLRQAEVIKGSWQPVGDLLIDSLQDHLEKVKALRGEIAPLKENV
Predicted Species
Rat (98%). Backed by our 100% Guarantee.
Isotype
IgG
Clonality
Polyclonal
Host
Rabbit
Gene
DMD
Purity
Immunogen affinity purified
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dystrophin (muscular dystrophy, Duchenne and Becker types), includes DXS142
dystrophin
Background
Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse.8 Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of BMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine.9 The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies. Monoclonal antibodies against defined regions10 of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.11
Limitations
This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.
FAQs for Dystrophin Antibody (NBP1-89953). (Showing 1 - 1 of 1 FAQs).
Do you know if this antibody has been used in paraffin or frozens section using immunofluorescence? Also, is there any cross-reactivity with mouse?
Yes this antibody has been validated for use in IHC-P in Human but we predict there will be cross reactivity to mouse based on the 96% homology. The human immunogen sequence homology is 96% homologous with the mouse protein.
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