Ultrastructure and molecular pathogenesis of epidermolysis bullosa

Shinkuma, Satoru, McMillan, James R. and Shimizu, Hiroshi (2011) Ultrastructure and molecular pathogenesis of epidermolysis bullosa. Clinics in Dermatology, 29 4: 412-419. doi:10.1016/j.clindermatol.2011.01.010


Author Shinkuma, Satoru
McMillan, James R.
Shimizu, Hiroshi
Title Ultrastructure and molecular pathogenesis of epidermolysis bullosa
Journal name Clinics in Dermatology   Check publisher's open access policy
ISSN 0738-081X
1879-1131
Publication date 2011
Sub-type Article (original research)
DOI 10.1016/j.clindermatol.2011.01.010
Open Access Status
Volume 29
Issue 4
Start page 412
End page 419
Total pages 8
Place of publication Philadelphia, PA, United States
Publisher Elsevier
Subject 2708 Dermatology
Abstract Epidermolysis bullosa (EB) is classified into the three major subtypes depending on the level of skin cleavage within the epidermal keratinocyte or basement membrane zone. Tissue separation occurs within the intraepidermal cytoplasm of the basal keratinocyte, through the lamina lucida, or in sublamina densa regions of the basal lamina (basement membrane) in EB simplex, junctional EB, and dystrophic EB, respectively. Transmission electron microscopy (TEM) is an effective method for determining the level of tissue separation and hemidesmosome (HD) and anchoring fibril morphology if performed by experienced operators, and has proven to be a powerful technique for the diagnosis of new EB patients. Recent advances in genetic and immunofluorescence studies have enabled us to diagnose EB more easily and with greater accuracy. This contribution reviews TEM findings in the EB subtypes and discusses the importance of observations in the molecular morphology of HD and basement membrane associated structures.
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Medicine Publications
 
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