Fibrillin microfibril structure identifies long-range effects of inherited pathogenic mutations affecting a key regulatory latent TGFβ-binding site

Autor: Alan R. F. Godwin, Rana Dajani, Xinyang Zhang, Jennifer Thomson, David F. Holmes, Christin S. Adamo, Gerhard Sengle, Michael J. Sherratt, Alan M. Roseman, Clair Baldock

Publikováno v: Nature Structural & Molecular Biology. 30:608-618

Genetic mutations in fibrillin microfibrils cause serious inherited diseases, such as Marfan syndrome and Weill–Marchesani syndrome (WMS). These diseases typically show major dysregulation of tissue development and growth, particularly in skeletal

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::400c67b7f71f6588859742122393a277
https://doi.org/10.1038/s41594-023-00950-8

Circadian control of the secretory pathway maintains collagen homeostasis

Autor: Antony Adamson, Joan Chang, David F. Holmes, Qing-Jun Meng, Joe Swift, Oliver E. Jensen, Ching-Yan Chloé Yeung, Adam Pickard, Venkatesh Mallikarjun, Karl E. Kadler, Tom Shearer, Ben Calverley, Helena Raymond-Hayling, Yinhui Lu, Richa Garva

Publikováno v: Chang, J, Garva, R, Pickard, A, Yeung, C Y C, Mallikarjun, V, Swift, J, Holmes, D, Calverley, B, Lu, Y, Adamson, A, Raymond-Hayling, H, Jensen, O, Shearer, T, Meng, Q-J & Kadler, K 2020, ' Circadian control of the secretory pathway maintains collagen homeostasis ', Nature Cell Biology, vol. 22, no. 1, pp. 74-86 . https://doi.org/10.1038/s41556-019-0441-z

Collagen is the most abundant secreted protein in vertebrates and persists throughout life without renewal. The permanency of collagen networks contrasts with both the continued synthesis of collagen throughout adulthood and the conventional transcri

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::291ccc1a5013b9e03b0a9c79b2965645
https://doi.org/10.1038/s41556-019-0441-z

A structure-based extracellular matrix expansion mechanism of fibrous tissue growth

Autor: Nicholas S Kalson, Yinhui Lu, Susan H Taylor, Tobias Starborg, David F Holmes, Karl E Kadler

Publikováno v: eLife, Vol 4 (2015)

Embryonic growth occurs predominately by an increase in cell number; little is known about growth mechanisms later in development when fibrous tissues account for the bulk of adult vertebrate mass. We present a model for fibrous tissue growth based o

Externí odkaz: https://doaj.org/article/f87b25cc9b8a4ff0a9a42fe3e1935505

Analysis of opticin binding to collagen fibrils identifies a single binding site in the gap region and a high specificity towards thin heterotypic fibrils containing collagens II, and XI or V/XI

Autor: Paul N. Bishop, Uwe Hansen, David F. Holmes, Peter Bruckner

Publikováno v: PLoS ONE
PLoS ONE, Vol 15, Iss 8, p e0234672 (2020)

Opticin is a class III member of the extracellular matrix small leucine-rich repeat protein/proteoglycan (SLRP) family found in vitreous humour and cartilage. It was first identified associated with the surface of vitreous collagen fibrils and severa

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a52451fd682ae1596fb4862acfd76294
https://pubmed.ncbi.nlm.nih.gov/32764753

Estrogen mediates acute elastic fibre homeostasis in skin

Autor: Joe Swift, Elaine Emmerson, Charis R Saville, David F. Holmes, Matthew J. Hardman, Brian Derby, Michael J. Sherratt

Remodelling of the dermal extracellular matrix makes a major contribution to skin fragility in the elderly. The peri-menopausal period in females is also associated with an age-like phenotype which can be reversed by hormone replacement therapy. This

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7687ad93ea00e23f2c1de7ca792d2f1d
https://doi.org/10.1101/728865