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.

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Products

Proteins (17)

Type

Over-Expression Lysate (12)

Recombinant (5)

Target

DMD / Dystrophin (17)

Publications

No (17)

Tag

GST (1)

His (2)

Myc-DDK (Flag) (14)

Species

Human (10)

Mouse (1)

Source

293T Cells (6)

E. coli (2)

HEK 293 Cells (8)

Wheat Germ Extract (1)

Purification

Purified (1)

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Human DMD / Dystrophin Protein (Recombinant Myc-DDK (Flag)) (Full Length) - LS-G68048

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

143 kDa

Price:

20 µg/$1,107

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Human DMD / Dystrophin Protein (Recombinant His) (aa3048-3328) - LS-G13921

Source:

E. coli

Tag:

His

Molecular Weight:

33.7 kDa

Price:

10 µg/$276; 50 µg/$412; 100 µg/$657; 200 µg/$822; 1 mg/$1,905; 500 µg/$1,455; 5 mg/$2,871; 2 mg/$2,090

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Mouse DMD / Dystrophin Protein (Recombinant His) (aa3059-3314) - LS-G13924

Source:

E. coli

Tag:

His

Molecular Weight:

30.8 kDa

Price:

10 µg/$279; 50 µg/$432; 100 µg/$690; 200 µg/$862; 1 mg/$1,962; 500 µg/$1,505; 5 mg/$3,011; 2 mg/$2,148

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Human DMD / Dystrophin Protein (Recombinant Myc-DDK (Flag)) (Full Length) - LS-G68047

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

70.6 kDa

Price:

20 µg/$1,107

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81313

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

70.2 kDa

Price:

20 µg/$215

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81314

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

72 kDa

Price:

20 µg/$215

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81315

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

68.8 kDa

Price:

20 µg/$215

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81316

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

39.1 kDa

Price:

20 µg/$150

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81318

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

143.6 kDa

Price:

20 µg/$215

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Human DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G81317

Source:

293T Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

70.6 kDa

Price:

20 µg/$215

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Human DMD / Dystrophin Protein (Recombinant GST) (Full Length) - LS-G28219

Source:

Wheat Germ Extract

Tag:

GST

Molecular Weight:

95.37 kDa

Price:

10 µg/$479; 25 µg/$670

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116933

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

72 kDa

Price:

100 µg/$710

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116932

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

70.2 kDa

Price:

100 µg/$710

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116934

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

68.8 kDa

Price:

100 µg/$710

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116935

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

70.6 kDa

Price:

100 µg/$710

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116938

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

143.6 kDa

Price:

100 µg/$710

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DMD / Dystrophin Protein (Over-Expression Lysate Myc-DDK (Flag)) (Full Length) - LS-G116936

Source:

HEK 293 Cells

Tag:

Myc-DDK (Flag)

Molecular Weight:

39.1 kDa

Price:

100 µg/$494

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