Keratins and epidermolysis bullosa simplex.

Abstract:

:Keratin intermediate filaments play an important role in maintaining the integrity of the skin structure. Understanding the importance of this subject is possible with the investigation of keratin defects in epidermolysis bullosa simplex (EBS). Nowadays, in addition to clinical criteria, new molecular diagnostic methods, such as next generation sequencing, can help to distinguish the subgroups of EBS more precisely. Because the most important and most commonly occurring molecular defects in these patients are the defects of keratins 5 and14 (KRT5 and KRT14), comprehending the nature structure of these proteins and their involved processes can be very effective in understanding the pathophysiology of this disease and providing new and effective therapeutic platforms to treat it. Here, we summarized the various aspects of the presence of KRT5 and KRT14 in the epidermis, their relation to the incidence and severity of EBS phenotypes, and the processes with which these proteins can affect them.

journal_name

J Cell Physiol

authors

Khani P,Ghazi F,Zekri A,Nasri F,Behrangi E,Aghdam AM,Mirzaei H

doi

10.1002/jcp.26898

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

289-297

issue

1

eissn

0021-9541

issn

1097-4652

journal_volume

234

pub_type

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