نتایج جستجو برای: pachyonychia congenita

تعداد نتایج: 3662  

Journal: :The Journal of dermatology 2006
Se-Woong Oh Moon Young Kim Jeong Sun Lee Soo-Chan Kim

Pachyonychia congenita type 2 (PC-2) is an autosomal dominant disorder characterized by hypertrophic nail dystrophy, focal keratoderma, multiple pilosebaceous cysts, and other features of ectodermal dysplasia. It has been demonstrated that PC-2 is caused by mutations in the keratin 17 and keratin 6b genes. In this report, we describe a missense mutation in the keratin 17 gene, M88T, in a Korean...

Journal: :The Journal of investigative dermatology 2010
Paul E Bowden

Twenty years have elapsed since keratin mutations were linked to cutaneous genodermatoses, and we now know that they cause 40 different genetic disorders. In this issue, Wilson et al. have identified KRT6C mutations in patients with focal palmoplantar keratoderma (FPPK), but debate concerning overlapping phenotypes between FPPK and pachyonychia congenita (PC) will continue because only one fami...

Journal: :Indian journal of dermatology, venereology and leprology 2008
Amirhushang Ehsani Fatemeh Moeineddin Ahmadreza Rajaee

A 10-month-old female presented with severe progressive wedge-shaped thickening and discoloration of all twenty nails. Further evaluations revealed palmoplantar keratoderma along with recurrent acral blisters causing residual crusted ulcers which were present during the past six months. Other findings included scalp kinky hair and dental caries. Past medical and family history had remarkable fi...

Journal: :The British journal of dermatology 2014
J Ofaiche S Duchatelet S Fraitag A Nassif J Nougué A Hovnanian

Sec. 107. Limitations on exclusive rights: Fair use Notwithstanding the provisions of sections 106 and 106A, the fair use of a copyrighted work, including such use by reproduction in copies or phonorecords or by any other means specified by that section, for purposes such as criticism, comment, news reporting, teaching (including multiple copies for classroom use), scholarship, or research, is ...

Journal: :Journal of Investigative Dermatology 2007

Journal: :Molecular therapy : the journal of the American Society of Gene Therapy 2010
Sancy A Leachman Robyn P Hickerson Mary E Schwartz Emily E Bullough Stephen L Hutcherson Kenneth M Boucher C David Hansen Mark J Eliason G Susan Srivatsa Douglas J Kornbrust Frances Jd Smith Wh Irwin McLean Leonard M Milstone Roger L Kaspar

The rare skin disorder pachyonychia congenita (PC) is an autosomal dominant syndrome that includes a disabling plantar keratoderma for which no satisfactory treatment is currently available. We have completed a phase Ib clinical trial for treatment of PC utilizing the first short-interfering RNA (siRNA)-based therapeutic for skin. This siRNA, called TD101, specifically and potently targets the ...

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