Linked Life Data

9389789

Source:http://linkedlifedata.com/resource/pubmed/id/9389789

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
5
pubmed:dateCreated
1997-12-15
pubmed:abstractText
The cytoplasmic domain of integrin beta 4, which contains four type III fibronectin-like motifs, seems to be involved in the regulation of the assembly of hemidesmosomes (HD) and, therefore, in cell adhesion. An in-frame deletion of 17 amino acids in the second fibronectin type III repeat of integrin beta 4 (delta 17-beta 4) has been associated with junctional epidermolysis bullosa with pyloric atresia (PA-JEB), a genetic disease characterized by altered HD and disadhesion of the epidermis. To determine the effect of deletion delta 17-beta 4 on HD assembly, we have examined the expression and localization of the HD components in the skin and cultured keratinocytes of a patient with PA-JEB, which express the mutated integrin beta 4. Our results show that the mutated beta 4 subunit associates with integrin alpha 6, but the resulting heterodimer does not induce nucleation of the bullous pemphigoid antigens BP180 and BP230, and that of the inner plaque component plectin/HD1, into hemidesmosomal structures. The integrity of the cytoplasmic tail of integrin beta 4 seems to be essential to the targeting and stabilization of plectin/HD1 and BP180 in HD, because transfection of a recombinant wild-type B4 cDNA in the delta 17-beta 4 PA-JEB keratinocytes restores the synthesis of a functional alpha 6/beta 4 heterodimer, which promotes the polarization of plectin/HD1 and BP180, to the basal aspect of the cells. Because in the transfected keratinocytes the distribution of BP230 remains diffuse in the cytoplasm, we suggest that the interaction between plectin/HD1 and integrin alpha 6 beta 4, followed by the association with BP180, constitutes the first step in the nucleation of the HD.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0023-6837
pubmed:author
pubmed:issnType
Print
pubmed:volume
77
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
459-68
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1997
pubmed:articleTitle
Hemidesmosome assembly assessed by expression of a wild-type integrin beta 4 cDNA in junctional epidermolysis bullosa keratinocytes.
pubmed:affiliation
Institut National de la Sante et de la Recherche Medicale, Faculté de Médecine, Hôpital de l'Archet 2, Nice, France.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't