TAILIEUCHUNG - Báo cáo khoa hoc : BRICHOS domain associated with lung fibrosis, dementia and cancer – a chaperone that prevents amyloid fibril formation?
REVIEW ARTICLE Hanna Willander1,2, EriThe BRICHOS domain was initially defined from sequence alignments of the Bri protein associated with familial dementia, chondromodulin associ-ated with chondrosarcoma and surfactant protein C precursor (proSP-C) associated with respiratory distress syndrome and interstitial lung disease (ILD). Today BRICHOS has been found in 12 protein Hermansson2, Jan Johansson1,2 and Jenny Presto1,2 1 Department of Anatomy, Physiology and Biochemistry, Swedish University of Agricultural Sciences, Uppsala, Sweden 2 KI-Alzheimer’s Disease Research Center, NVS Department, Karolinska Institutet, Stockholm, Sweden Keywords Alzheimer’s disease; Bri; chaperone; dementia; lung fibrosis, proSP-C, protein misfolding Correspondence J. Presto, KI-Alzheimer’s disease research center, NVS department, Karolinska Institutet, Stockholm, Sweden Fax:. | IFEBS Journal REVIEW ARTICLE BRICHOS domain associated with lung fibrosis dementia and cancer - a chaperone that prevents amyloid fibril formation Hanna Willander1 2 Erik Hermansson2 Jan Johansson1 2 and Jenny Presto1 2 1 Department of Anatomy Physiology and Biochemistry Swedish University of AgriculturalSciences Uppsala Sweden 2 KI-Alzheimer s Disease Research Center NVS Department Karolinska Institutet Stockholm Sweden Keywords Alzheimer s disease Bri chaperone dementia lung fibrosis proSP-C protein misfolding Correspondence J. Presto KI-Alzheimer s disease research center NVS department Karolinska Institutet Stockholm Sweden Fax 46 8 585 836 10 Tel 46 8 585 839 03 E-mail Received 14 April 2011 revised 31 May 2011 accepted 7 June 2011 doi The BRICHOS domain was initially defined from sequence alignments of the Bri protein associated with familial dementia chondromodulin associated with chondrosarcoma and surfactant protein C precursor proSP-C associated with respiratory distress syndrome and interstitial lung disease ILD . Today BRICHOS has been found in 12 protein families. Mutations in the Bri2 and proSP-C genes result in familial dementia and ILD respectively and both these conditions are associated with amyloid formation. Amyloid is of great medical relevance as it is found in several major incurable diseases like Alzheimer s and Parkinson s disease and diabetes mellitus. Work on recombinant BRICHOS domains and transfected cells indicate that BRICHOS is a chaperone domain that during biosynthesis binds to precursor protein regions with high b-sheet propensities thereby preventing them from amyloid formation. Regions prone to form b-sheets are present in all BRICHOS-containing precursor proteins and are probably eventually released by proteolytic cleavage generating different peptides with largely unknown bioactivities. Recombinant BRICHOS domains from Bri2 and proSP-C have been found to efficiently prevent .
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