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Titolo:
FIBRILLIN-1 IN HUMAN CARTILAGE - DEVELOPMENTAL EXPRESSION AND FORMATION OF SPECIAL BANDED FIBERS
Autore:
KEENE DR; JORDAN CD; REINHARDT DP; RIDGWAY CC; ONO RN; CORSON GM; FAIRHURST M; SUSSMAN MD; MEMOLI VA; SAKAI LY;
Indirizzi:
SHRINERS HOSP CRIPPLED CHILDRENS,3101 SW SAM JACKSON PK RD PORTLAND OR 97201 SHRINERS HOSP CRIPPLED CHILDRENS PORTLAND OR 97201 OREGON HLTH SCI UNIV,DEPT BIOCHEM & MOL BIOL PORTLAND DORSET ENGLAND DARTMOUTH HITCHCOCK MED CTR,DEPT PATHOL LEBANON NH 03766
Titolo Testata:
The Journal of histochemistry and cytochemistry
fascicolo: 8, volume: 45, anno: 1997,
pagine: 1069 - 1082
SICI:
0022-1554(1997)45:8<1069:FIHC-D>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
CONNECTIVE-TISSUE MICROFIBRILS; MARFAN-SYNDROME; EXTRACELLULAR MICROFIBRILS; COMPONENT; GENE; MUTATIONS; DISORDER; SEQUENCE;
Keywords:
FIBRILLIN; CARTILAGE; MICROFIBRILS; MARFANS SYNDROME; SPECIAL BANDED FIBERS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
26
Recensione:
Indirizzi per estratti:
Citazione:
D.R. Keene et al., "FIBRILLIN-1 IN HUMAN CARTILAGE - DEVELOPMENTAL EXPRESSION AND FORMATION OF SPECIAL BANDED FIBERS", The Journal of histochemistry and cytochemistry, 45(8), 1997, pp. 1069-1082

Abstract

The molecular basis for Marfan's syndrome (MS), a heritable disorder of connective tissue, is now known to reside in mutations in FBN1, thegene for fibrillin-1. Classic phenotypic manifestations of MS includeseveral skeletal abnormalities associated primarily with overgrowth of long bones. As a first step towards understanding how mutations in FBN1 result in skeletal abnormalities, the developmental expression of fibrillin-1 (Fib-1) in human skeletal tissues is documented using immunohistochemistry and monoclonal antibodies demonstrated here to be specific for Fib-l. At around 10-11 weeks of fetal gestation, Fib-l is limited in tissue distribution to the loose connective tissue surrounding skeletal muscle and tendon in developing limbs. By 16 weeks, Fib-l is widely expressed in developing limbs and digits, especially in the perichondrium, but it is apparently absent within cartilage matrix. Fib-1 appears as a loose meshwork of fibers within cartilage matrix by 20weeks of fetal gestation. Until early adolescence, Fib-1 forms loose bundles of microfibrils within cartilage. However, by late adolescence, broad banded fibers composed of Fib-l are found accumulated pericellularly within cartilage. Because these fibers can be extracted from cartilage using dissociative conditions, we postulate that they are laterally packed and crosslinked microfibrils. On the basis of these findings, we suggest that the growth-regulating function of Fib-l may reside persistently within the perichondrium. In addition, the accumulationof special laterally crosslinked Fib-1 microfibrils around chondrocytes during late adolescence suggests that growth-regulating activities may also be performed by Fib-l at these sites.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 10:04:53