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Titolo:
Selective insolubility of alpha-Synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model
Autore:
Kahle, PJ; Neumann, M; Ozmen, L; Muller, V; Odoy, S; Okamoto, N; Jacobsen, H; Iwatsubo, T; Trojanowski, JQ; Takahashi, H; Wakabayashi, K; Bogdanovic, N; Riederer, P; Kretzschmar, HA; Haass, C;
Indirizzi:
Univ Munich, Dept Biochem, Lab Alzheimers & Parkinsons Dis Res, D-80336 Munich, Germany Univ Munich Munich Germany D-80336 sons Dis Res, D-80336 Munich, Germany Univ Munich, Dept Neuropathol, D-80336 Munich, Germany Univ Munich Munich Germany D-80336 Neuropathol, D-80336 Munich, Germany F Hoffmann La Roche & Co Ltd, Pharma Res Genomics, CH-4002 Basel, Switzerland F Hoffmann La Roche & Co Ltd Basel Switzerland CH-4002 asel, Switzerland Univ Tokyo, Grad Sch Pharmaceut Sci, Dept Neuropathol & Neurosci, Tokyo, Japan Univ Tokyo Tokyo Japan t Sci, Dept Neuropathol & Neurosci, Tokyo, Japan Univ Penn, Sch Med, Dept Pathol & Lab Med, Ctr Neurodegenerat Dis Res, Philadelphia, PA 19104 USA Univ Penn Philadelphia PA USA 19104 t Dis Res, Philadelphia, PA 19104 USA Niigata Univ, Dept Pathol, Brain Res Inst, Niigata, Japan Niigata Univ Niigata Japan Dept Pathol, Brain Res Inst, Niigata, Japan Hirosaki Univ, Sch Med, Inst Brain Sci, Dept Neuropathol, Hirosaki, Aomori036, Japan Hirosaki Univ Hirosaki Aomori Japan 036 athol, Hirosaki, Aomori036, Japan Huddinge Brain Bank, Geriatr Sect, Dept Clin Neurosci, Huddinge, Sweden Huddinge Brain Bank Huddinge Sweden ept Clin Neurosci, Huddinge, Sweden Univ Wurzburg, Dept Psychiat, Wurzburg, Germany Univ Wurzburg Wurzburg Germany rzburg, Dept Psychiat, Wurzburg, Germany Univ Wurzburg, Natl Parkinson Fdn Ctr Excellence Res Labs, Wurzburg, Germany Univ Wurzburg Wurzburg Germany r Excellence Res Labs, Wurzburg, Germany
Titolo Testata:
AMERICAN JOURNAL OF PATHOLOGY
fascicolo: 6, volume: 159, anno: 2001,
pagine: 2215 - 2225
SICI:
0002-9440(200112)159:6<2215:SIOAIH>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
BRAIN IRON ACCUMULATION; A-BETA COMPONENT; HALLERVORDEN-SPATZ-DISEASE; MULTIPLE SYSTEM ATROPHY; PARKINSONS-DISEASE; IN-VITRO; NACP/ALPHA-SYNUCLEIN; NEURODEGENERATIVE DISORDERS; SPORADIC PARKINSONS; PRECURSOR PROTEIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
54
Recensione:
Indirizzi per estratti:
Indirizzo: Kahle, PJ Univ Munich, Dept Biochem, Lab Alzheimers & Parkinsons Dis Res, Schillerstr 44, D-80336 Munich, Germany Univ Munich Schillerstr 44 Munich Germany D-80336 nich, Germany
Citazione:
P.J. Kahle et al., "Selective insolubility of alpha-Synuclein in human Lewy body diseases is recapitulated in a transgenic mouse model", AM J PATH, 159(6), 2001, pp. 2215-2225

Abstract

alpha -Synuclein (alpha -SYN) is deposited in intraneuronal cytoplasmic inclusions (Lewy bodies, LBs) characteristic for Parkinson's disease (PD) andLB dementias. alpha -SYN forms LB-like fibrils in vitro, in contrast to its homologue beta -SYN. Here we have investigated the solubility of SYNs in human LB diseases and in transgenic mice expressing human wild-type and PD-associated mutant [A30P]alpha -SYN driven by the brain neuron-specific promoter, Thy1. Distinct alpha -SYN species were detected in the detergent-insoluble fractions from brains of patients with PD, dementia with LBs, and neurodegeneration with brain iron accumulation type 1 (formerly known as Hallervorden-Spatz disease). Using the same extraction method, detergent-insolubility of human alpha -SYN was observed in brains of transgenic mice. In contrast, neither endogenous mouse alpha -SYN nor beta -SYN were detected in detergent-insoluble fractions from transgenic mouse brains. The nonamyloidogenic beta -SYN was incapable of forming insoluble fibrils because amino acids 73 to 83 in the central region of alpha -SYN are absent in beta -SYN. Inconclusion, the specific accumulation of detergent-insoluble alpha -SYN intransgenic mice recapitulates a pivotal feature of human LB diseases.

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Documento generato il 30/03/20 alle ore 12:21:04