Document Detail


A review of genetic disorders of hypopigmentation: lessons learned from the biology of melanocytes.
MedLine Citation:
PMID:  19555431     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Inherited diseases of pigmentation were among the first traits studied in humans because of their easy recognition. The discovery of genes that regulate melanocytic development and function and the identification of disease-causative mutations have greatly improved our understanding of the molecular basis of pigmentary genodermatoses and their underlying pathogenetic mechanisms. Pigmentation mutants can account for hypo-/amelanosis, with or without altered melanocyte number, resulting in different phenotypes, such as Waardenburg syndrome, piebaldism, Hermansky-Pudlak syndrome, Chediak-Higashi syndrome, oculocutaneous albinism and Griscelli syndrome. In this review, we summarize the basic concepts of melanocyte biology and discuss how molecular defects in melanocyte development and function can result in the development of hypopigmentary hereditary skin diseases.
Authors:
Clio Dessinioti; Alexander J Stratigos; Dimitris Rigopoulos; Andreas D Katsambas
Publication Detail:
Type:  Journal Article; Review     Date:  2009-06-23
Journal Detail:
Title:  Experimental dermatology     Volume:  18     ISSN:  1600-0625     ISO Abbreviation:  Exp. Dermatol.     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-21     Completed Date:  2009-12-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9301549     Medline TA:  Exp Dermatol     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  741-9     Citation Subset:  IM    
Affiliation:
Department of Dermatology, A. Sygros Hospital, University of Athens, Athens, Greece.
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MeSH Terms
Descriptor/Qualifier:
Humans
Hypopigmentation / genetics*
Keratinocytes / physiology
Melanins / biosynthesis
Melanocytes / physiology*
Chemical
Reg. No./Substance:
0/Melanins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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