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DMD / Dystrophin

dystrophin

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.

Gene Name: dystrophin
Synonyms: DMD, BMD, CMD3B, DXS206, Dystrophin, DXS230, DXS239, DXS269, DXS142, DXS268, DXS164, DXS270, DXS272
Target Sequences: NM_000109 P11532 P11532

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Products
Proteins (17)
Over-Expression Lysate (12)
Recombinant (5)
DMD / Dystrophin (17)
No (17)
GST (1)
His (2)
Myc-DDK (Flag) (14)
Human (10)
Mouse (1)
293T Cells (6)
E. coli (2)
HEK 293 Cells (8)
Wheat Germ Extract (1)
Purified (1)
DMD / Dystrophin Protein - Purified recombinant protein DMD was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
143 kDa
20 µg/$1,024
DMD / Dystrophin Protein - Recombinant Dystrophin By SDS-PAGE
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E. coli
His
33.7 kDa
10 µg/$266; 50 µg/$393; 100 µg/$627; 200 µg/$784; 1 mg/$1,781; 500 µg/$1,328; 5 mg/$3,819
DMD / Dystrophin Protein - Purified recombinant protein DMD was analyzed by SDS-PAGE gel and Coomassie Blue Staining
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HEK 293 Cells
Myc-DDK (Flag)
70.6 kDa
20 µg/$1,005
DMD / Dystrophin Protein - 12.5% SDS-PAGE of human DMD stained with Coomassie Blue
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Wheat Germ Extract
GST
95.37 kDa
10 µg/$469; 25 µg/$630
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
70.2 kDa
100 µg/$607
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
68.8 kDa
100 µg/$607
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
70.6 kDa
100 µg/$607
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
72 kDa
100 µg/$607
Select
HEK 293 Cells
Myc-DDK (Flag)
143.6 kDa
100 µg/$607
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
HEK 293 Cells
Myc-DDK (Flag)
39.1 kDa
100 µg/$458
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
72 kDa
20 µg/$200
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
70.2 kDa
20 µg/$200
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
68.8 kDa
20 µg/$200
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
70.6 kDa
20 µg/$200
DMD / Dystrophin Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
Select
293T Cells
Myc-DDK (Flag)
39.1 kDa
20 µg/$130
Select
293T Cells
Myc-DDK (Flag)
143.6 kDa
20 µg/$200
DMD / Dystrophin Protein - Recombinant Dystrophin By SDS-PAGE
Select
E. coli
His
30.8 kDa
10 µg/$269; 50 µg/$412; 100 µg/$658; 200 µg/$822; 1 mg/$1,847; 500 µg/$1,372; 5 mg/$3,985
Viewing 1-17 of 17 product results


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PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).