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Titolo:
Cases of Alzheimer's disease due to deletion of exon 9 of the presenilin-1gene show an unusual but characteristic beta-amyloid pathology known as 'cotton wool' plaques
Autore:
Mann, DMA; Takeuchi, A; Sato, S; Cairns, NJ; Lantos, PL; Rossor, MN; Haltia, M; Kalimo, H; Iwatsubo, T;
Indirizzi:
Univ Manchester, Dept Med, Clin Neurosci Res Grp, Manchester M13 9PT, Lancs, England Univ Manchester Manchester Lancs England M13 9PT M13 9PT, Lancs, England Univ Tokyo, Dept Neuropathol & Neurosci, Tokyo, Japan Univ Tokyo Tokyo Japan Tokyo, Dept Neuropathol & Neurosci, Tokyo, Japan Univ Tokyo, Inst Med Sci, Ctr Human Genome, Tokyo, Japan Univ Tokyo Tokyo Japan yo, Inst Med Sci, Ctr Human Genome, Tokyo, Japan Inst Psychiat, Dept Neuropathol, Brain Bank, London SE5 8AF, England Inst Psychiat London England SE5 8AF Brain Bank, London SE5 8AF, England Natl Hosp Neurol & Neurosurg, Dept Neurol, London WC1N 3BG, England Natl Hosp Neurol & Neurosurg London England WC1N 3BG n WC1N 3BG, England Univ Helsinki, Dept Pathol, Helsinki, Finland Univ Helsinki Helsinki Finland Helsinki, Dept Pathol, Helsinki, Finland Univ Helsinki, Cent Hosp, Helsinki, Finland Univ Helsinki Helsinki Finland v Helsinki, Cent Hosp, Helsinki, Finland Univ Turku, Dept Pathol, Turku, Finland Univ Turku Turku FinlandUniv Turku, Dept Pathol, Turku, Finland
Titolo Testata:
NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
fascicolo: 3, volume: 27, anno: 2001,
pagine: 189 - 196
SICI:
0305-1846(200106)27:3<189:COADDT>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROTEIN A-BETA; GAMMA-SECRETASE ACTIVITY; SPASTIC PARAPARESIS; MONOCLONAL-ANTIBODIES; PRECURSOR PROTEIN; MUTATIONS; DEPOSITION; ENDOPROTEOLYSIS; VARIANT; CATENIN;
Keywords:
Alzheimer's disease; beta-amyloid; mutation; presenilin-1 gene; senile plaque;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
34
Recensione:
Indirizzi per estratti:
Indirizzo: Mann, DMA Univ Manchester, Dept Med, Clin Neurosci Res Grp, Stopford Bldg,Oxford Rd,Manchester M13 9PT, Lancs, England Univ Manchester Stopford Bldg,Oxford Rd Manchester Lancs England M13 9PT
Citazione:
D.M.A. Mann et al., "Cases of Alzheimer's disease due to deletion of exon 9 of the presenilin-1gene show an unusual but characteristic beta-amyloid pathology known as 'cotton wool' plaques", NEUROP AP N, 27(3), 2001, pp. 189-196

Abstract

The pattern of deposition of amyloid beta protein (A beta) was investigated, using the monoclonal antibodies BA27 and BC05 detecting the C-terminal species A beta (40) and A beta (42(43)), in six cases of Alzheimer's disease(AD) due to deletions in exon 9 of PS-1 gene. These cases are characterized histologically by the presence of very large rounded plaques within the frontal cortex, known as 'cotton wool' plaques, composed of both A beta (40)and A beta (42(43)) that are relatively free from neuritic changes and glial cell components, and usually devoid of a compact amyloid core. In the cerebellum the plaques are almost entirely of a compact type, again composed of A beta (40) and A beta (42(43)), with only few diffuse A beta (42(43)) containing plaques. The area fraction of A beta (40), and the ratio between A beta (40) and A beta (42(43)), in frontal cortex was significantly higherthan that seen in other cases of AD due to different PS-1 mutations, or incases of sporadic AD, all of similar APO E genotype. The area fractions ofA beta (42(43)), however, did not significantly differ between these threegroups. The unusual nature of the A beta deposition in these cases may reflect the uniqueness of the mutation, which results in a failure to constitutively cleave the PS-1 holoprotein into its active form, and the effect this might have on APP trafficking and catabolism.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/04/20 alle ore 12:57:35