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Cultured keratinocytes from plectin/HD1-deficient epidermolysis bullosa simplex showed altered ability of adhesion to the matrix.

Abstract：

:Epidermolysis bullosa simplex associated with late onset of muscular dystrophy has been found to show defective expression of plectin, an intracytoplasmic protein in hemidesmosomes. In this report, we examined ability of cell-to-matrix attachment of cultured keratinocytes derived from a case with this disease by various cell biological methods, and compared it to that of normal keratinocytes. In cell adhesion assay, the patient keratinocytes showed more prominent short-time cell adhesion than normal keratinocytes. In contrast, the patient keratinocytes could be detached much easier than normal keratinocytes in cell detachment assay by treatment with dispase. In phagokinetic track assay, no apparent difference of cell migration was observed between the patient and normal keratinocytes. These results indicate that plectin-deficiency may up-regulate short-term cell contact and reduce stable cell-matrix adhesion at the epidermal basement membrane zone.

journal_name

J Dermatol Sci

journal_title

Journal of dermatological science

authors

Kurose K,Mori O,Hachisuka H,Shimizu H,Owaribe K,Hashimoto T

doi

10.1016/s0923-1811(00)00100-6

subject

Has Abstract

pub_date

2000-12-01 00:00:00

pages

184-9

issue

3

eissn

0923-1811

issn

1873-569X

pii

S0923181100001006

journal_volume

24

pub_type

杂志文章

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