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Understanding Inherited Cylindromas Clinical Implications of Gene Discovery

Lookup NU author(s): Dr Kirsty Hodgson, Dr Neil Rajan

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Abstract

Cylindromas are cutaneous tumors first described in the medical literature over 150 years ago. They are now known to occur on an inherited basis as a result of mutations in the tumor-suppressor gene CYLD. The discovery of this gene has provided novel insights into this rare skin tumor syndrome. As well as enabling genetic counseling of affected patients, the knowledge of CYLD function has led to steps toward development of novel therapeutics, with CYLD-regulated signaling pathways as the target for this approach.


Publication metadata

Author(s): Dubois A, Hodgson K, Rajan N

Publication type: Article

Publication status: Published

Journal: Dermatologic Clinics

Year: 2017

Volume: 35

Issue: 1

Pages: 61-71

Print publication date: 01/01/2017

Online publication date: 25/11/2016

Acceptance date: 02/04/2016

ISSN (print): 0733-8635

ISSN (electronic): 1558-0520

Publisher: Elsevier

URL: http://dx.doi.org/10.1016/j.det.2016.08.002

DOI: 10.1016/j.det.2016.08.002


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