Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
GENE TARGETING AT THE MOUSE CYTOKERATIN-10 LOCUS - SEVERE SKIN FRAGILITY AND CHANGES OF CYTOKERATIN EXPRESSION IN THE EPIDERMIS
Autore:
PORTER RM; LEITGEB S; MELTON DW; SWENSSON O; EADY RAJ; MAGIN TM;
Indirizzi:
UNIV BONN,INST GENET,ABT MOL GENET,ROMERSTR 164 D-53117 BONN GERMANY ST THOMAS HOSP,ST JOHNS INST DERMATOL,DEPT CELL PATHOL LONDON SE1 7EHENGLAND UNIV EDINBURGH,INST CELL & MOLEC BIOL EDINBURGH EH9 3JR MIDLOTHIAN SCOTLAND
Titolo Testata:
The Journal of cell biology
fascicolo: 5, volume: 132, anno: 1996,
pagine: 925 - 936
SICI:
0021-9525(1996)132:5<925:GTATMC>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
EPIDERMOLYSIS-BULLOSA SIMPLEX; INTERMEDIATE FILAMENTS; KERATIN EXPRESSION; CONSENSUS SEQUENCE; EPITHELIAL-CELLS; POINT MUTATIONS; GROWTH-FACTOR; TERMINAL END; COILED-COIL; ROD DOMAIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
99
Recensione:
Indirizzi per estratti:
Citazione:
R.M. Porter et al., "GENE TARGETING AT THE MOUSE CYTOKERATIN-10 LOCUS - SEVERE SKIN FRAGILITY AND CHANGES OF CYTOKERATIN EXPRESSION IN THE EPIDERMIS", The Journal of cell biology, 132(5), 1996, pp. 925-936

Abstract

Bullous congenital ichthyosiform erythroderma (BCIE) is a dominantly inherited blistering skin disorder caused by point mutations in the suprabasal cytokeratins 1 or 10. Targeting the murine cytokeratin 10 gene in ES cells resulted in mice with different phenotypes in the homozygotes and heterozygotes; both of which exhibit similarities to specific clinical characteristics of BCIE. Homozygotes suffered from severe skin fragility and died shortly after birth. Heterozygotes were apparently unaffected at birth, but developed hyperkeratosis with age. In both genotypes, aggregation of cytokeratin intermediate filaments, changes in cytokeratin expression, and alterations in the program of epidermal differentiation were observed. In addition we demonstrate, for the first time, the existence of the murine equivalent of human cytokeratin 16.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 06:36:31