2026-06-14T03:44:45Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/126852025-05-08T08:50:05Zcom_20.500.12105_2052com_20.500.12105_2051col_20.500.12105_19627
Repisalud
author
Rábano, Alberto
author
Guerrero Márquez, Carmen
author
Juste, Ramón A
author
Geijo, María V
author
Calero, Miguel
2021-04-19T15:23:50Z
2021-04-19T15:23:50Z
2021-03-10
Biomolecules . 2021 Mar 10;11(3):413.
10.3390/biom11030413
2218-273X
Biomolecules
33802224
http://hdl.handle.net/20.500.12105/12685
Human prion and non-prion neurodegenerative diseases share pathogenic mechanisms and neuropathological features. The lesion profile of a particular entity results from specific involvement of vulnerable neuron populations and connectivity circuits by a pathogenic protein isoform with strain-like properties. The lesion profile of the medial temporal lobe (MTL) was studied in postmortem tissue of 143 patients with human prion disease (HPD) including sporadic, genetic, and acquired forms. Most cases (90%) were classified according to PrPres type and/or PRNP codon 129 status, in addition to a full neuropathological profile. Mixed histotypes represented 29.4% of total sporadic Creutzfeldt-Jakob disease (sCJD) cases. An intensity score of involvement including spongiosis and astrogliosis was determined for the amygdala, presubiculum, subiculum, entorhinal cortex, CA1 to CA4 sectors of the hippocampal cortex, and dentate gyrus. Connectivity hubs within the MTL presented the highest scores. Diverse lesion profiles were obtained for different types and subtypes of HPD. Impact of mixed PrPres types on the MTL lesion profile was higher for sCJDMV2K cases than in other histotypes. Differences between MTL profiles was globally consistent with current evidence on specific strains in HPD. These results may be relevant for the analysis of possible strain effects in focal non-prion neurodegenerative conditions limited to the MTL.
eng
Human prion diseases
Medial temporal lobe
Neurodegenerative diseases
Prion strains
Medial Temporal Lobe Involvement in Human Prion Diseases: Implications for the Study of Focal Non Prion Neurodegenerative Pathology.
research article
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MedialTemporalLobeInvolvement_2021.pdf
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https://repisalud.isciii.es/bitstreams/86316bf0-b9eb-451a-b77b-49f681169353/download
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MedialTemporalLobeInvolvement_2021.pdf.txt