We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Hemidesmosomes Show Abnormal Association with the Keratin Filament Network in Junctional Forms of Epidermolysis Bullosa.
- Authors
McMillan, James R.; McGrath, John A.; Tidman, Michael J.; Eady, Robin A. J.
- Abstract
Junctional epidermolysis bullosa is a group of hereditary bullous disorders resulting from defects in several hemidesmosome‐anchoring filament components. Because hemidesmosomes are involved not only in keratinocyte‐extracellular matrix adherence, but also in normal anchorage of keratin intermediate filaments to the basal keratinocyte membrane, we questioned whether this intracellular function of hemidesmosomes was also perturbed in junctional epidermolysis bullosa. We used quantitative electron microscopic methods to assess certain morphologic features of hemidesmosome–keratin intermediate filaments interactions in skin from normal subjects (n = 11) and from patients with different forms of junctional epidermolysis bullosa (n = 13). In addition, skin from patients with autosomal recessive epidermolysis bullosa simplex with plectin defects (n = 3) or with autosomal recessive dystrophic epidermolysis bullosa (n = 4) were included as controls. Values were expressed as a percentage of the total number of hemidesmosomes counted. In normal skin 83.3% ± 3.3 (SEM) hemidesmosomes were associated with keratin intermediate filaments and 90.1% ± 1.9 had inner plaques. In Herlitz junctional epidermolysis bullosa (laminin 5 abnormalities, n = 4) these values were reduced to 45.3% ± 11.5 (p < 0.001; analysis of variance) and 50.3% ± 12.8 (p < 0.001), respectively. In junctional epidermolysis bullosa with pyloric atresia (α6β4 abnormalities, n = 3) the values were also reduced [41.8% ± 7.0 (p < 0.001) and 44.5% ± 5.7 (p < 0.001), respectively]. In the non‐Herlitz group (laminin 5 mutations, n = 3) the counts were 66.7% ± 7.1 (p > 0.05) and 70.5% ± 8.5 (p < 0.05), and in skin from patients with bullous pemphigoid antigen 2 mutations (n = 3) the counts were 54.3% ± 13.8 (p < 0.01) and 57.1% ± 13.9 (p < 0.01). In epidermolysis bullosa simplex associated with plectin mutations the values were...
- Subjects
EPIDERMOLYSIS bullosa; HEMIDESMOSOMES
- Publication
Journal of Investigative Dermatology, 1998, Vol 110, Issue 2, p132
- ISSN
0022-202X
- Publication type
Article
- DOI
10.1046/j.1523-1747.1998.00102.x