Sex Matters: Interfering with the Oxidative Stress Response in Pachyonychia Congenita
نویسندگان
چکیده
منابع مشابه
Oxidative stress and dysfunctional NRF2 underlie pachyonychia congenita phenotypes.
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...
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Pachyonychia congenita is a rare genodermatosis that can affect the larynx. Laryngeal obstruction is very unusual with only a few cases reported. A 2-year-old girl presented with typical clinical features of pachyonychia congenita shortly after birth. At age 9 months, following an upper respiratory infection, she developed stridor and hoarseness and was found to have severe laryngeal obstructio...
متن کاملSensitivity to Oxidative Stress: Sex Matters
The excessive production of free radicals in organism and the imbalance between the concentration of these and the antioxidant defences has been related to the process of aging. It has been postulated that oxidative processes and antioxidant defence can bee sex related. Besides, we have noticed that at old age 60% of male mice developed hepatocellular tumors which were absent in females. Thus, ...
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There are currently no specific treatments for pachyonychia congenita (PC). Available treatments generally are directed at specific manifestations of the disorder, and an effective treatment plan must recognize that different patients are more or less troubled by different manifestations of the disease. Treatment for all aspects of PC has been less than completely satisfactory. Very few studies...
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ژورنال
عنوان ژورنال: Journal of Investigative Dermatology
سال: 2018
ISSN: 0022-202X
DOI: 10.1016/j.jid.2017.12.017