نتایج جستجو برای: pachyonychia congenita

تعداد نتایج: 3662  

Journal: :Indian Journal of Dermatology, Venereology and Leprology 2008

Journal: :The Journal of investigative dermatology 2011
Marta García Fernando Larcher Robyn P Hickerson Eulalia Baselga Sancy A Leachman Roger L Kaspar Marcela Del Rio

Molecular characterization and assessment of therapeutic outcomes for inherited cutaneous disorders requires faithful preclinical models. In this study we report the establishment of two different skin-humanized pachyonychia congenita (PC) model systems, based on permanent engraftment of bioengineered skin equivalents generated from patient skin cells onto immunodeficient mice. Using keratinocy...

Journal: :The Journal of clinical investigation 2016
Michelle L Kerns Jill M C Hakim Rosemary G Lu Yajuan Guo Andreas Berroth Roger L Kaspar Pierre A Coulombe

Palmoplantar keratoderma (PPK) are debilitating lesions that arise in individuals with pachyonychia congenita (PC) and feature upregulation of danger-associated molecular patterns and skin barrier regulators. The defining features of PC-associated PPK are reproduced in mice null for keratin 16 (Krt16), which is commonly mutated in PC patients. Here, we have shown that PPK onset is preceded by o...

1999
U. Villante S. Lepidi P. Francia M. Vellante A. Meloni R. P. Lepping F. Mariani

Pc3 geomagnetic ®eld ̄uctuations detected at low latitude (L'Aquila, Italy) during the passage of a high velocity solar wind stream, characterized by variable interplanetary magnetic ®eld conditions, are analyzed. Higher frequency resonant ̄uctuations and lower frequency phenomena are simultaneously observed; the intermittent appearance and the variable frequency of the longer period modes can ...

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