Literature DB >> 17476352

Cutaneous mosaicism: right before our eyes.

Jorge Frank1, Rudolf Happle.   

Abstract

Autosomal recessive cutaneous disorders, including various types of epidermolysis bullosa (EB), usually manifest shortly after birth. The clinical course of these diseases is often characterized by severe complications, limited therapeutic options, and a poor prognosis. A study by Pasmooij et al. reported in this issue of the JCI unravels the molecular mechanisms by which germline mutations in non-Herlitz junctional EB can be corrected in vivo by multiple spontaneously occurring somatic mutational events, a phenomenon known as revertant mosaicism (see the related article beginning on page 1240). These insights open new avenues of thinking for the design of future gene therapy strategies for skin diseases.

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Year:  2007        PMID: 17476352      PMCID: PMC1857254          DOI: 10.1172/JCI32111

Source DB:  PubMed          Journal:  J Clin Invest        ISSN: 0021-9738            Impact factor:   14.808


  26 in total

1.  Mosaic analysis in Caenorhabditis elegans.

Authors:  J Yochem; M Sundaram; E A Bucher
Journal:  Methods Mol Biol       Date:  2000

2.  Dohi Memorial Lecture. New aspects of cutaneous mosaicism.

Authors:  Rudolf Happle
Journal:  J Dermatol       Date:  2002-11       Impact factor: 4.005

3.  Genetic mosaic techniques for studying Drosophila development.

Authors:  Seth S Blair
Journal:  Development       Date:  2003-11       Impact factor: 6.868

4.  Genetics and dermatology or if I were to rewrite Cockayne's Inherited Abnormalities of the Skin.

Authors:  V A McKusick
Journal:  J Invest Dermatol       Date:  1973-06       Impact factor: 8.551

5.  Revertant mosaicism in junctional epidermolysis bullosa due to multiple correcting second-site mutations in LAMB3.

Authors:  Anna M G Pasmooij; Hendri H Pas; Maria C Bolling; Marcel F Jonkman
Journal:  J Clin Invest       Date:  2007-05       Impact factor: 14.808

Review 6.  The molecular genetics of the genodermatoses: progress to date and future directions.

Authors:  A D Irvine; W H I McLean
Journal:  Br J Dermatol       Date:  2003-01       Impact factor: 9.302

7.  A transposon-like element in the deletion-prone region of the dystrophin gene.

Authors:  A Pizzuti; M Pieretti; R G Fenwick; R A Gibbs; C T Caskey
Journal:  Genomics       Date:  1992-07       Impact factor: 5.736

Review 8.  Translational benefits from research on rare genodermatoses.

Authors:  John A McGrath
Journal:  Australas J Dermatol       Date:  2004-05       Impact factor: 2.875

9.  Spontaneous reversion of novel Lesch-Nyhan mutation by HPRT gene rearrangement.

Authors:  T P Yang; J T Stout; D S Konecki; P I Patel; R L Alford; C T Caskey
Journal:  Somat Cell Mol Genet       Date:  1988-05

10.  Characterization of revertant muscle fibers in Duchenne muscular dystrophy, using exon-specific monoclonal antibodies against dystrophin.

Authors:  L T Thanh; T M Nguyen; T R Helliwell; G E Morris
Journal:  Am J Hum Genet       Date:  1995-03       Impact factor: 11.025

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  1 in total

1.  Somitic disruption of GNAS in chick embryos mimics progressive osseous heteroplasia.

Authors:  Dana M Cairns; Robert J Pignolo; Tomoya Uchimura; Tracy A Brennan; Carter M Lindborg; Meiqi Xu; Frederick S Kaplan; Eileen M Shore; Li Zeng
Journal:  J Clin Invest       Date:  2013-07-25       Impact factor: 14.808

  1 in total

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