Literature DB >> 10204788

Dystrophin and utrophin do not play crucial roles in nonmuscle tissues in mice.

J A Rafael1, J I Trickett, A C Potter, K E Davies.   

Abstract

The loss of full-length dystrophin from skeletal muscle leads to the clinical features of Duchenne muscular dystrophy. Both Dp71, a C-terminal dystrophin isoform, and the dystrophin-related protein, utrophin, are present at high levels in many nonmuscle tissues. To investigate the roles of these proteins in nonmuscle tissues, mice were generated null for utrophin, and deficient in all dystrophin isoforms. These mice reach adulthood and do not appear to have any devastating pathology in nonmuscle tissues.

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Year:  1999        PMID: 10204788     DOI: 10.1002/(sici)1097-4598(199904)22:4<517::aid-mus14>3.0.co;2-6

Source DB:  PubMed          Journal:  Muscle Nerve        ISSN: 0148-639X            Impact factor:   3.217


  5 in total

1.  Dystroglycan does not contribute significantly to kidney development or function, in health or after injury.

Authors:  George Jarad; Jeffrey W Pippin; Stuart J Shankland; Jordan A Kreidberg; Jeffrey H Miner
Journal:  Am J Physiol Renal Physiol       Date:  2011-01-05

2.  Molecular characterization of a de novo 6q24.2q25.3 duplication interrupting UTRN in a patient with arthrogryposis.

Authors:  Anne-Claude Tabet; Azzedine Aboura; Marion Gérard; Marion Pilorge; Céline Dupont; Jean-François Gadisseux; Nadège Hervy; Eva Pipiras; Andrée Delahaye; Samia Kanafani; Alain Verloes; Brigitte Benzacken; Catalina Betancur
Journal:  Am J Med Genet A       Date:  2010-07       Impact factor: 2.802

3.  Renal involvement in the pathogenesis of mineral and bone disorder in dystrophin-deficient mdx mouse.

Authors:  Eiji Wada; Takayuki Hamano; Isao Matsui; Mizuko Yoshida; Yukiko K Hayashi; Ryoichi Matsuda
Journal:  J Physiol Sci       Date:  2019-05-11       Impact factor: 2.781

4.  Low dystrophin levels increase survival and improve muscle pathology and function in dystrophin/utrophin double-knockout mice.

Authors:  Maaike van Putten; Margriet Hulsker; Courtney Young; Vishna D Nadarajah; Hans Heemskerk; Louise van der Weerd; Peter A C 't Hoen; Gert-Jan B van Ommen; Annemieke M Aartsma-Rus
Journal:  FASEB J       Date:  2013-03-04       Impact factor: 5.191

5.  Marginal level dystrophin expression improves clinical outcome in a strain of dystrophin/utrophin double knockout mice.

Authors:  Dejia Li; Yongping Yue; Dongsheng Duan
Journal:  PLoS One       Date:  2010-12-20       Impact factor: 3.240

  5 in total

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