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Titolo:
Functions of surfactant proteins B and C
Autore:
Weaver, TE; Conkright, JJ;
Indirizzi:
Childrens Hosp, Med Ctr, Div Pulm Biol, Cincinnati, OH 45229 USA ChildrensHosp Cincinnati OH USA 45229 ulm Biol, Cincinnati, OH 45229 USA
Titolo Testata:
ANNUAL REVIEW OF PHYSIOLOGY
, volume: 63, anno: 2001,
pagine: 555 - 578
SICI:
0066-4278(2001)63:<555:FOSPBA>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
POLYPEPTIDE SP-C; CONGENITAL ALVEOLAR PROTEINOSIS; PULMONARY EPITHELIAL-CELLS; RAT TYPE-II; SECONDARY STRUCTURE; LUNG SURFACTANT; IN-VITRO; DISULFIDE BRIDGES; LAMELLAR BODIES; DEFICIENT MICE;
Keywords:
type II epithelial cell; lung development; transgenic mice; respiratory distress syndrome; structure-function;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
137
Recensione:
Indirizzi per estratti:
Indirizzo: Weaver, TE Childrens Hosp, Med Ctr, Div Pulm Biol, Cincinnati, OH 45229 USA Childrens Hosp Cincinnati OH USA 45229 incinnati, OH 45229 USA
Citazione:
T.E. Weaver e J.J. Conkright, "Functions of surfactant proteins B and C", ANN R PHYSL, 63, 2001, pp. 555-578

Abstract

SP-B is the only surfactant-associated protein absolutely required for postnatal lung function and survival. Complete deficiency of SP-B in mice and humans results in lethal, neonatal respiratory distress syndrome and is characterized by a virtual absence of lung compliance, highly disorganized lamellar bodies, and greatly diminished levels of SP-C mature peptide; in contrast, lung structure and function in SP-C null mice is normal. This review attempts to integrate recent findings in humans and transgenic mice with the results of in vitro studies to provide a better understanding of the functions of SP-B and SP-C and the structural basis for their actions.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 18/09/20 alle ore 17:01:03