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Mouse Models for Blistering Skin Disorders

Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses...

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Detalles Bibliográficos
Autores principales: Ganeshan, Radhika, Chen, Jiangli, Koch, Peter J.
Formato: Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2879910/
https://www.ncbi.nlm.nih.gov/pubmed/20585602
http://dx.doi.org/10.1155/2010/584353
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author Ganeshan, Radhika
Chen, Jiangli
Koch, Peter J.
author_facet Ganeshan, Radhika
Chen, Jiangli
Koch, Peter J.
author_sort Ganeshan, Radhika
collection PubMed
description Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses on mice that lack the desmosomal cadherins desmoglein 3 or desmocollin 3 in stratified epithelia. A comparison of the phenotypes observed in these mouse lines is provided and the relationship between the mutant mouse phenotypes and human diseases, in particular pemphigus vulgaris, is discussed. Furthermore, we will discuss the advantages and potential limitations of genetically engineered mouse lines in our ongoing quest to understand blistering skin diseases.
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spelling pubmed-28799102010-06-28 Mouse Models for Blistering Skin Disorders Ganeshan, Radhika Chen, Jiangli Koch, Peter J. Dermatol Res Pract Review Article Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses on mice that lack the desmosomal cadherins desmoglein 3 or desmocollin 3 in stratified epithelia. A comparison of the phenotypes observed in these mouse lines is provided and the relationship between the mutant mouse phenotypes and human diseases, in particular pemphigus vulgaris, is discussed. Furthermore, we will discuss the advantages and potential limitations of genetically engineered mouse lines in our ongoing quest to understand blistering skin diseases. Hindawi Publishing Corporation 2010 2010-05-10 /pmc/articles/PMC2879910/ /pubmed/20585602 http://dx.doi.org/10.1155/2010/584353 Text en Copyright © 2010 Radhika Ganeshan et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Review Article
Ganeshan, Radhika
Chen, Jiangli
Koch, Peter J.
Mouse Models for Blistering Skin Disorders
title Mouse Models for Blistering Skin Disorders
title_full Mouse Models for Blistering Skin Disorders
title_fullStr Mouse Models for Blistering Skin Disorders
title_full_unstemmed Mouse Models for Blistering Skin Disorders
title_short Mouse Models for Blistering Skin Disorders
title_sort mouse models for blistering skin disorders
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2879910/
https://www.ncbi.nlm.nih.gov/pubmed/20585602
http://dx.doi.org/10.1155/2010/584353
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