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Dystrophin 产品

(Dystrophin (DMD))

Categories

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].

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Featured Dystrophin Categories

Dystrophin 抗体

High quality antibodies with extensive validation data.

Dystrophin ELISA试剂盒

Reliable ELISA kits for a wide range of species.

Dystrophin 蛋白

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended Dystrophin 抗体

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Application WB, IHC, IF
Validations
  • (5)
Cat. No. ABIN1679546
Quantity 100 μg
Datasheet Datasheet
Reactivity Human, Mouse, Rat
Application WB, IHC, IF
Validations
  • (4)
Cat. No. ABIN7008615
Quantity 60 μL
Datasheet Datasheet
Reactivity Human
Application WB, IP, ELISA
Validations
  • (4)
Cat. No. ABIN515027
Quantity 100 μg
Datasheet Datasheet

Recommended Dystrophin ELISA试剂盒

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Rat
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6966439
Quantity 96 tests
Datasheet Datasheet
Reactivity Mouse
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6966438
Quantity 96 tests
Datasheet Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
  • (1)
Cat. No. ABIN6955484
Quantity 96 tests
Datasheet Datasheet

Recommended Dystrophin 蛋白

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1351717
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Escherichia coli (E. coli)
Validations
Cat. No. ABIN7420834
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Source Escherichia coli (E. coli)
Validations
Cat. No. ABIN7394210
Quantity 100 μg
Datasheet Datasheet

Latest Publications for our Dystrophin Products

Stay, Miterko, Arancillo, Lin, Sillitoe: "In vivo cerebellar circuit function is disrupted in an mdx mouse model of Duchenne muscular dystrophy." in: Disease models & mechanisms, Vol. 13, Issue 2, (2019) (PubMed).

Mondello, Cardia, Bartoloni, Asmundo, Ventura Spagnolo: "Immunohistochemical study on dystrophin expression in CAD-related sudden cardiac death: a marker of early myocardial ischaemia." in: International journal of legal medicine, Vol. 132, Issue 5, pp. 1333-1339, (2018) (PubMed).

Aoyama, Kawase, Bando, Monji, Murohara et al.: "Dipeptidyl Peptidase 4 Inhibition Alleviates Shortage of Circulating Glucagon-Like Peptide-1 in Heart Failure and Mitigates Myocardial Remodeling and Apoptosis via the Exchange Protein Directly ..." in: Circulation. Heart failure, Vol. 9, Issue 1, pp. e002081, (2016) (PubMed).

Annese, Corsi, Ruggieri, Tamma, Marinaccio, Picocci, Errede, Specchia, De Luca, Frassanito, Desantis, Vacca, Ribatti, Nico: "Isolation and characterization of neural stem cells from dystrophic mdx mouse." in: Experimental cell research, Vol. 343, Issue 2, pp. 190-207, (2016) (PubMed).

Puchert, Adams, Linke, Engele: "Evidence for the involvement of the CXCL12 system in the adaptation of skeletal muscles to physical exercise." in: Cellular signalling, Vol. 28, Issue 9, pp. 1205-15, (2016) (PubMed).

Kawase, Bando, Nishimura, Aoyama, Monji, Murohara: "A dipeptidyl peptidase-4 inhibitor ameliorates hypertensive cardiac remodeling via angiotensin-II/sodium-proton pump exchanger-1 axis." in: Journal of molecular and cellular cardiology, Vol. 98, pp. 37-47, (2016) (PubMed).

Hosur, Kavirayani, Riefler, Carney, Lyons, Gott, Cox, Shultz: "Dystrophin and dysferlin double mutant mice: a novel model for rhabdomyosarcoma." in: Cancer genetics, Vol. 205, Issue 5, pp. 232-41, (2012) (PubMed).

Nguyen, Ginjaar, van Ommen, Morris: "Monoclonal antibodies for dystrophin analysis. Epitope mapping and improved binding to SDS-treated muscle sections." in: The Biochemical journal, Vol. 288 ( Pt 2), pp. 663-8, (1993) (PubMed).

Morris, Ellis, Nguyen: "A quantitative ELISA for dystrophin." in: Journal of immunological methods, Vol. 161, Issue 1, pp. 23-8, (1993) (PubMed).

Synonyms and alternative names related to Dystrophin

dystrophin (DMD), dystrophin (LOC708073), dystrophin, gene 1 L homeolog (dmd.1.L), dystrophin (LOC465557), dystrophin, gene 1 (dmd.1), dystrophin (dmd), dystrophin, muscular dystrophy (Dmd), dystrophin (Dmd), Dystrophin (Dys), BMD, cb664, CG7240, CG7243, CG7344, CG17750, CG31175, CG34157, CMD3B, det, DKFZp459C1629, DKFZp468A1620, DLP, DLP1, DLP2, DLP3, DLP186, DMD, Dmd, dmd, DmDLP, dmDLP, dmDp186, DmDYS, dmDys, Dmel\\CG34157, DNADMD1, Dp71, Dp117, Dp186, Dp205, Dp427, DXS142, DXS164, DXS206, DXS230, DXS239, DXS268, DXS269, DXS270, DXS272, DXSmh7, DXSmh9, dys, GI3046716, IDLP, im:6911785, mdx, MGC79631, MGC83347, pke, RATDMD, zfDYS, zgc:110165

Protein level used designations for Dystrophin

  • dystrophin (muscular dystrophy, Duchenne and Becker types)
  • dystrophin
  • Duchenne muscular dystrophy
  • dystrophin isoform Dp116
  • sap
  • sapje
  • dystrophin Dp71 isoform
  • X-linked muscular dystrophy
  • RNDNADMD1
  • apodystrophin-3
  • apodystrophin-I
  • dystrophin transcript variant Dp71e
  • dystrophin, muscular dystrophy
  • CG34157-PA
  • CG34157-PB
  • CG34157-PC
  • CG34157-PD
  • CG34157-PE
  • CG34157-PF
  • CG34157-PG
  • CG34157-PH
  • CG34157-PI
  • CG34157-PJ
  • CG34157-PK
  • CG34157-PL
  • Dys-PA
  • Dys-PB
  • Dys-PC
  • Dys-PD
  • Dys-PE
  • Dys-PF
  • Dys-PG
  • Dys-PH
  • Dys-PI
  • Dys-PJ
  • Dys-PK
  • Dys-PL
  • Dystrophin-like protein 1
  • Dystrophin-like protein 186
  • Dystrophin-like protein 2
  • Dystrophin-like protein 3
  • detached
  • dystrophin Dp186
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