Dystrophin Recombinant Rabbit Monoclonal Antibody [JF1-022]
Catalog# ET1702-98
Dystrophin Recombinant Rabbit Monoclonal Antibody [JF1-022]
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WB
-
IHC-P
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IHC-Fr
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Human
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Mouse
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Rat
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unconjugated
Overview
Product Name
Dystrophin Recombinant Rabbit Monoclonal Antibody [JF1-022]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Synthetic peptide within Human Dystrophin aa 3651-3685 / 3685.
Species Reactivity
Human, Mouse, Rat
Validated Applications
WB, IHC-P, IHC-Fr
Target Molecular Weight
Predicted band size: 427 kDa
Positive Control
Mouse skeletal muscle tissue lysate, Mouse heart tissue lysate, Rat skeletal muscle tissue lysate, Rat heart tissue lysate, human heart tissue, mouse heart tissue, rat heart tissue, human striated muscle tissue, mouse skeletal muscle tissue, rat heart tissue.
Conjugation
unconjugated
Clone Number
JF1-022
RRID
Product Features
Form
Liquid
Concentration
1 mg/mL.(The concentration of this product may be batch-dependent)
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
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1:1,000
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IHC-P
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1:200-1:1,000
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IHC-Fr
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1:200
Target
Function
Dystrophin is a rod-shaped cytoplasmic protein, and a vital part of a protein complex that connects the cytoskeleton of a muscle fiber to the surrounding extracellular matrix through the cell membrane. This complex is variously known as the costamere or the dystrophin-associated protein complex (DAPC). Many muscle proteins, such as α-dystrobrevin, syncoilin, synemin, sarcoglycan, dystroglycan, and sarcospan, colocalize with dystrophin at the costamere. It has a molecular weight of 427 kDa. Dystrophin is coded for by the DMD gene – the largest known human gene, covering 2.4 megabases (0.08% of the human genome) at locus Xp21. The primary transcript in muscle measures about 2,100 kilobases and takes 16 hours to transcribe; the mature mRNA measures 14.0 kilobases. The 79-exon muscle transcript codes for a protein of 3685 amino acid residues. Spontaneous or inherited mutations in the dystrophin gene can cause different forms of muscular dystrophy, a disease characterized by progressive muscular wasting. The most common of these disorders caused by genetic defects in dystrophin is Duchenne muscular dystrophy.
Background References
1. Fortunato F et al. The DMD gene and therapeutic approaches to restore dystrophin. Neuromuscul Disord. 2021 Oct
2. Bönnemann CG et al. Dystrophin Immunity after Gene Therapy for Duchenne\'s Muscular Dystrophy. N Engl J Med. 2023 Jun
Tissue Specificity
Expressed in muscle fibers accumulating in the costameres of myoplasm at the sarcolemma. Expressed in brain, muscle, kidney, lung and testis. Most tissues contain transcripts of multiple isoforms. Isoform 15: Only isoform to be detected in heart and liver and is also expressed in brain, testis and hepatoma cells.
Subcellular Location
Cell membrane, sarcolemma, Cytoplasm, cytoskeleton, Postsynaptic cell membrane.
Synonyms
BMD antibody
CMD3B antibody
DMD antibody
DMD_HUMAN antibody
Duchenne muscular dystrophy protein antibody
Dystrophin antibody
Muscular dystrophy Duchenne and Becker types antibody
Images
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☑ Relative expression (RE)
Western blot analysis of Dystrophin on different lysates with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/1,000 dilution.
Lane 1: Mouse skeletal muscle tissue lysate
Lane 2: Mouse heart tissue lysate
Lane 3: Mouse liver tissue lysate (negative)
Lane 4: Rat skeletal muscle tissue lysate
Lane 5: Rat heart tissue lysate
Lane 6: Rat liver tissue lysate (negative)
Lysates/proteins at 40 µg/Lane.
Predicted band size: 427 kDa
Observed band size: 427 kDa
Exposure time: 4 seconds; ECL: K1801;
3-8% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ET1702-98) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
Immunohistochemical analysis of paraffin-embedded human heart tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded mouse heart tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/1,000 dilution.
The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0) (high pressure) for 2 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded rat heart tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/1,000 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded human striated muscle tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/400 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/400 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded mouse skeletal muscle tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/1,000 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunohistochemical analysis of paraffin-embedded rat skeletal muscle tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/1,000 dilution.
The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0) (high pressure) for 2 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET1702-98) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Immunofluorescence analysis of frozen mouse skeletal muscle tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0) for about 2 minutes in microwave oven. The tissues were blocked in 10% negative goat serum for 1 hour at room temperature, washed with PBS, and then probed with the primary antibody (ET1702-98, green) at 1/200 dilution overnight at 4 ℃, washed with PBS. Goat Anti-Rabbit IgG H&L (iFluor™ 488, HA1121) was used as the secondary antibody at 1/1,000 dilution. Nuclei were counterstained with DAPI (blue). -
Immunofluorescence analysis of frozen rat heart tissue with Rabbit anti-Dystrophin antibody (ET1702-98) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0) for about 2 minutes in microwave oven. The tissues were blocked in 10% negative goat serum for 1 hour at room temperature, washed with PBS, and then probed with the primary antibody (ET1702-98, green) at 1/200 dilution overnight at 4 ℃, washed with PBS. Goat Anti-Rabbit IgG H&L (iFluor™ 488, HA1121) was used as the secondary antibody at 1/1,000 dilution. Nuclei were counterstained with DAPI (blue).
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"