Výsledky vyhledávání

Regulation of fibrillin carboxy-terminal furin processing by N-glycosylation, and association of amino- and carboxy-terminal sequences

Autor: J L, Ashworth, V, Kelly, M J, Rock, C A, Shuttleworth, C M, Kielty

Publikováno v: Journal of Cell Science. 112:4163-4171

The molecular mechanisms of fibrillin assembly into microfibrils are poorly understood. In this study, we investigated human fibrillin-1 carboxy-terminal processing and assembly using a recombinant approach. Processing of carboxy-terminal fibrillin-1

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bb678f2af920e0024d1311c8d5b6562e
https://doi.org/10.1242/jcs.112.22.4163

Zobrazit plný text záznamu

Fibrillin: From Microfibril Assembly to Biomechanical Function

Autor: Clair Baldock, Cay M. Kielty, Jane Ashworth, C.A. Shuttleworth, M. J. Rock, David Lee

Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long–range elasticity. Their biological importance is emphasized by the linkage of fibrillin mutations

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7ff72b7188987b389a2a2eac8c134a49
https://doi.org/10.1017/cbo9780511546327.007

Zobrazit plný text záznamu

Fibrillin degradation by matrix metalloproteinases: implications for connective tissue remodelling

Autor: J L, Ashworth, G, Murphy, M J, Rock, M J, Sherratt, S D, Shapiro, C A, Shuttleworth, C M, Kielty

Publikováno v: The Biochemical journal. 340

Fibrillin is the principal structural component of the 10-12 nm diameter elastic microfibrils of the extracellular matrix. We have previously shown that both fibrillin molecules and assembled microfibrils are susceptible to degradation by serine prot

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::c55a881cf68fa75655a547ce474e51b0
https://pubmed.ncbi.nlm.nih.gov/10229672

Zobrazit plný text záznamu

Aerosol delivery and safety of recombinant human deoxyribonuclease in young children with cystic fibrosis: a bronchoscopic study. Pulmozyme Pediatric Broncoscopy Study Group

Autor: J S, Wagener, M J, Rock, M M, McCubbin, S D, Hamilton, C A, Johnson, R C, Ahrens

Publikováno v: The Journal of pediatrics. 133(4)

The purpose of this study was to assess the delivery to the lungs and the short-term safety of recombinant human deoxyribonuclease (rhDNase, Pulmozyme) in children with cystic fibrosis younger than 5 years of age compared with older children. Patient

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::c80982f1e08db72eb109ab56de72f458
https://pubmed.ncbi.nlm.nih.gov/9787685

Zobrazit plný text záznamu

A family study describing second cousins with cystic fibrosis and no common ancestor who is a carrier

Autor: Vicki M. Park, M T Knight, M J Rock, M E Smith

Publikováno v: Journal of medical genetics. 32(5)

We describe an extended pedigree in which second cousins are affected with cystic fibrosis (CF). Since the degree of relationship of the affected subjects is 1/32, common descent of one CF mutation was expected when the DNA laboratory undertook mutat

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0b17dc7eb37750b962ea8b72f7f2fbd2
https://pubmed.ncbi.nlm.nih.gov/8782060

Zobrazit plný text záznamu

The diagnostic utility of bronchoalveolar lavage in immunocompetent children with unexplained infiltrates on chest radiograph

Autor: M J, Rock

Publikováno v: Pediatrics. 95(3)

To assess the diagnostic utility of bronchoalveolar lavage (BAL) in a heterogeneous group of immunocompetent children with unexplained infiltrates on chest radiograph.This was a retrospective case series.The setting was a tertiary care referral cente

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::c9f964a1370faa0fe3462d65b4094f47
https://pubmed.ncbi.nlm.nih.gov/7862475

Zobrazit plný text záznamu

Vyhledávací nástroje:

Upřesnit hledání