Year: 2008
Type: article
Abstract: Propagation of transmissible spongiform encephalopathies is associated with the conversion of normal prion protein, PrP C , into a misfolded, oligomeric form, PrP Sc .While the high-resolution structure of PrP C is well characterized, the structural properties of PrP Sc remain elusive.Here we used mass spectroscopic analysis of hydrogen/deuterium backbone amide exchange (HXMS) to examine the structure of amyloid fibrils formed by the recombinant human prion protein corresponding to residues 90-231 (PrP90-231), a misfolded form recently reported to be infectious in transgenic mice overexpressing PrP C .Table 1.1.Human amyloid (or aggregate) proteins and their associated diseases.Protein Associated disease Serum amyloid protein A Secondary systemic amyloidosis Apolipoprotein A-I Familial amyloid polyneuropathy Type II Apolipoprotein A-II Familial amyloid polyneuropathy Type III Transthyretin Familial amyloid polyneuropathy Type I Lactoferrin Corneal amyloidosis Fibrinogen Fibrinogen amyloidosis Lysozyme Lyzozyme amyloidosis Keratin Cutaneous amyloidosis Tau Alzheimer's disease, frontotemporal dementia Amylin Type II diabetes Calcitonin Medullary carcinoma of the thyroid Prolactin Aging pituitary prolactinomas Insulin Insulin-related amyloid Atrial natriuretic factor Atrial amyloidosis Gelsolin Finnish hereditary amyloidosis Cystatin C Icelandic hereditary cerebral amyloid angiopathy Immunoglobulin light chains (κ and λ) Primary systemic amyloidosis, amyloidosis associated with multiple myeloma Immunoglobulin heavy chain Primary systemic amyloidosis β2-Microglobulin Hemodialysis-related amyloidosis Kerato-epithelin Corneal dystrophy Lactadherin (Medin) Aortic medial amyloidosis Amyloid-β Alzheimer's disease, cerebral amyloid angiopathy Prion protein Spongiform encephalopathies Amyloid British British familial dementia Amyloid Danish Danish familial dementia α-Synuclein Parkinson's disease
Author: Xiaojun Lu
Institution: Case Western Reserve University
Language: English
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: green
OpenAlex ID: W2300227099