MyBioSource

antibody

Mouse Anti-Human Dystrophin Monoclonal Antibody (Clone SPM499)

MBS301208

7 ml

280 USD

monoclonal

mouse

human, dog, mouse

immunohistochemistry, immunohistochemistry - paraffin section

MBS301208

Antibody

Mouse Anti-Human Dystrophin Monoclonal Antibody (Clone SPM499)

Dystrophin

dystrophin Dp427c isoform; Dystrophin; dystrophin; dystrophin; N/A

DMD; DMD; BMD; CMD3B; MRX85; DXS142; DXS164; DXS206; DXS230; DXS239; DXS268; DXS269; DXS270; DXS272; N/A

DMD_HUMAN

Monoclonal

IgG1/k

SPM499

Mouse

Human. Predicted Reactivity: Chicken, Dog, Mouse, Pig

3677

7.0 ml pre-diluted mouse monoclonal antibody purified by protein A/G in TBS/1% BSA buffer pH 7.6 with less than 0.1% sodium azide.

Store at 2-8 degree C. Do not freeze. The user must validate any other storage conditions. When properly stored, the reagent is stable to the date indicated on the label. Do not use the reagent beyond the expiration date. There are no definitive signs to indicate instability of this product; therefore, positive and negative controls should be tested simultaneously with unknown specimens. If unexpected results are observed which cannot be explained by variations in laboratory procedures and a problem with the reagent is suspected, contact Technical Support.

Immunohistochemistry (IHC) Paraffin

Immunohistochemistry Procedure . Specimen Preparation: Formalin-fixed, paraffin-embedded tissues are suitable for use with this primary antibody. Deparaffinization: Deparaffinize slides using xylene or xylene alternative and graded alcohols. Antibody Dilution: If using the concentrate format of this product, dilute the antibody 1:50. The dilutions are estimates; actual results may differ because of variability in methods and protocols. Antigen Retrieval: Boil tissue section in 10mM citrate buffer, pH 6.0 for 10 min followed by cooling at room temperature for 20 min. Primary Antibody Incubation: Incubate for 30 minutes at room temperature. Slide Washing: Slides must be washed in between steps. Rinse slides with PBS/0.05% Tween. Visualization: Detect the antibody as instructed by the instructions provided with the visualization system. Immunohistochemistry Dilution: Ready To Use. IHC Positive Control: Skeletal Muscle

Immunogen: Synthetic peptide corresponding to C-terminus of human dystrophin. Control Tissue: Skeletal Muscle. Cellular Localization: Membrane. Entrez Gene Code: 1756. Pathway: Muscle Markers

Dystrophin is a member of the spectrin/alpha-actinin family of actin-binding, triple helix rod-containing proteins. It is absent or greatly reduced in individuals with the X-linked recessive Duchenne's muscular dystrophy disorder, as well as in mice with the mdx (murine muscular dystrophy) mutation. This antibody is highly specific to dystrophin and shows no cross-reaction with C-protein (an isoform of alpha-actinin), alpha-actin, or human muscle spectrin.

5032281

NP_000100.2

NM_000109.3

427 kDa

Arrhythmogenic Right Ventricular Cardiomyopathy Pathway 672454!!Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway 117293!!Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway 116129!!Dilated Cardiomyopathy Pathway 121494!!Dilated Cardiomyopathy Pathway 121285!!Extracellular Matrix Organization Pathway 576262!!Hypertrophic Cardiomyopathy (HCM) Pathway 114229!!Hypertrophic Cardiomyopathy (HCM) Pathway 106591!!Muscle Contraction Pathway 106261!!Non-integrin Membrane-ECM Interactions Pathway 833810

The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq, Jul 2008]

7 ml

280 USD