Literature DB >> 26513728

Chemical chaperone therapy, a new strategy for genetic skin fragility disorders.

Jean Christopher Chamcheu1, Imtiaz A Siddiqui1, Hasan Mukhtar1.   

Abstract

Entities:  

Keywords:  chemical chaperone-based therapy; genetic skin diseases; keratin gene mutation; keratin genodermatoses; protein misfolding; skin fragility; stress-induced keratin aggregation

Mesh：

Substances：

Year:  2016        PMID： 26513728      PMCID： PMC5697139          DOI： 10.1111/exd.12893

Source DB:  PubMed          Journal:  Exp Dermatol        ISSN： 0906-6705            Impact factor:   3.960


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  10 in total

1.  Immortalized keratinocytes derived from patients with epidermolytic ichthyosis reproduce the disease phenotype: a useful in vitro model for testing new treatments.

Authors:  J C Chamcheu; I Pihl-Lundin; C E Mouyobo; T Gester; M Virtanen; A Moustakas; H Navsaria; A Vahlquist; H Törmä
Journal:  Br J Dermatol       Date:  2011-02       Impact factor: 9.302

2.  Gene expression analysis of an epidermolysis bullosa simplex Dowling-Meara cell line by subtractive hybridization: recapitulation of cellular differentiation, migration and wound healing.

Authors:  Martin Wagner; Helmut Hintner; Johann W Bauer; Kamil Onder
Journal:  Exp Dermatol       Date:  2011-12-14       Impact factor: 3.960

3.  Reduction in keratin aggregates in epidermolysis bullosa simplex keratinocytes after pretreatment with trimethylamine N-oxide.

Authors:  Mbarka Bchetnia; Jacynthe Lacroix; Tarik Farez; Miriam Larouche; Julie Powell; Catherine McCuaig; Audrey Dupéré; Charles Morin; Valérie Legendre-Guillemin; Catherine Laprise
Journal:  Exp Dermatol       Date:  2015-09-15       Impact factor: 3.960

4.  The chemical chaperone trimethylamine N-oxide ameliorates the effects of mutant keratins in cultured cells.

Authors:  D Lee; D Santos; H Al-Rawi; A M McNeill; E L Rugg
Journal:  Br J Dermatol       Date:  2008-07-01       Impact factor: 9.302

Review 5.  Keratin gene mutations in disorders of human skin and its appendages.

Authors:  Jean Christopher Chamcheu; Imtiaz A Siddiqui; Deeba N Syed; Vaqar M Adhami; Mirjana Liovic; Hasan Mukhtar
Journal:  Arch Biochem Biophys       Date:  2010-12-19       Impact factor: 4.013

Review 6.  Progress towards genetic and pharmacological therapies for keratin genodermatoses: current perspective and future promise.

Authors:  Jean Christopher Chamcheu; Gary S Wood; Imtiaz A Siddiqui; Deeba N Syed; Vaqar M Adhami; Joyce M Teng; Hasan Mukhtar
Journal:  Exp Dermatol       Date:  2012-07       Impact factor: 3.960

Review 7.  How do keratinizing disorders and blistering disorders overlap?

Authors:  Takahiro Hamada; Daisuke Tsuruta; Shunpei Fukuda; Norito Ishii; Kwesi Teye; Sanae Numata; Teruki Dainichi; Tadashi Karashima; Chika Ohata; Minao Furumura; Takashi Hashimoto
Journal:  Exp Dermatol       Date:  2012-10-08       Impact factor: 3.960

8.  Imbalance of intermediate filament component keratin 14 contributes to increased stress signalling in epidermolysis bullosa simplex.

Authors:  Martin Wagner; Andrea Trost; Helmut Hintner; Johann W Bauer; Kamil Onder
Journal:  Exp Dermatol       Date:  2013-04       Impact factor: 3.960

9.  Characterization of immortalized human epidermolysis bullosa simplex (KRT5) cell lines: trimethylamine N-oxide protects the keratin cytoskeleton against disruptive stress condition.

Authors:  Jean Christopher Chamcheu; Elizabeth Pavez Lorié; Baki Akgul; Elin Bannbers; Marie Virtanen; Luke Gammon; Aristidis Moustakas; Harshad Navsaria; Anders Vahlquist; Hans Törmä
Journal:  J Dermatol Sci       Date:  2009-01-20       Impact factor: 4.563

10.  Chemical chaperone ameliorates pathological protein aggregation in plectin-deficient muscle.

Authors:  Lilli Winter; Ilona Staszewska; Eva Mihailovska; Irmgard Fischer; Wolfgang H Goldmann; Rolf Schröder; Gerhard Wiche
Journal:  J Clin Invest       Date:  2014-03       Impact factor: 14.808
  10 in total
  1 in total

1.  Genotype-phenotype analysis of LMNA-related diseases predicts phenotype-selective alterations in lamin phosphorylation.

Authors:  Eric W Lin; Graham F Brady; Raymond Kwan; Alexey I Nesvizhskii; M Bishr Omary
Journal:  FASEB J       Date:  2020-05-15       Impact factor: 5.191
  1 in total

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