Article
Immortalized keratinocytes derived from patients with epidermolytic ichthyosis reproduce the disease phenotype: a useful in vitro model for testing new treatments.
The British journal of dermatology - 1 Feb 2011
Chamcheu J C, Pihl-Lundin I, Mouyobo C E, Gester T, Virtanen M, Moustakas A, Navsaria H, Vahlquist A, Törmä H
Abstract excerpt
BACKGROUND: Epidermolytic ichthyosis (EI) is a skin fragility disorder caused by mutations in genes encoding suprabasal keratins 1 and 10. While the aetiology of EI is known, model systems are needed for pathophysiological studies and development of novel therapies. OBJECTIVES: To generate immortalized keratinocyte lines from patients with EI for studies of EI cell pathology and the effects of chemical chaperones...
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