Tuberous Sclerosis (tsc2 /-) Model Eker Rats Reveals Extensive Neuronal Loss with Microglial Invasion and Vascular Remodeling Related to Brain Neoplasia
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023752%3A_____%2F20%3A43920092" target="_blank" >RIV/00023752:_____/20:43920092 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11120/20:43919409
Result on the web
<a href="https://link.springer.com/article/10.1007%2Fs13311-019-00812-6" target="_blank" >https://link.springer.com/article/10.1007%2Fs13311-019-00812-6</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s13311-019-00812-6" target="_blank" >10.1007/s13311-019-00812-6</a>
Alternative languages
Result language
angličtina
Original language name
Tuberous Sclerosis (tsc2 /-) Model Eker Rats Reveals Extensive Neuronal Loss with Microglial Invasion and Vascular Remodeling Related to Brain Neoplasia
Original language description
Tuberous sclerosis complex (TSC) is a genetic disorder characterized by frequent noncancerous neoplasia in the brain, which can induce a range of severe neuropsychiatric symptoms in humans, resulting from out of control tissue growth. The causative spontaneous loss-of-function mutations have been also identified in rats. Herein, we studied histopathological and molecular changes in brain lesions of the Eker rat model carrying germline mutation of the tsc2 gene, predisposed to multiple neoplasias. Predominant subcortical tumors were analyzed, along with a rare form occurring within the pyriform lobe. The uniform composition of lesions supports the histochemical parity of malformations, with immunofluorescence data supporting their neuroglial origin. Massive depletion of mature neurons and axonal loss were evident within lesions, with occasional necrotic foci implying advanced stage of pathology. Enrichment of mesenchymal-derived cell markers with hallmarks of neurogenesis and active microglia imply enhanced cell proliferation, with local immune response. The depletion of capillaries within the core was complemented by the formation of dense mesh of nascent vessels at the interface of neoplasia with healthy tissue, implying largescale vascular remodeling. Taken as a whole, these findings present several novel features of brain tumors in Eker rat model, rendering it suitable for studies of the pathobiology and progression of primary brain tumors, with therapeutic interventions.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
10620 - Other biological topics
Result continuities
Project
<a href="/en/project/LO1611" target="_blank" >LO1611: Sustainability for The National Institute of Mental Health</a><br>
Continuities
P - Projekt vyzkumu a vyvoje financovany z verejnych zdroju (s odkazem do CEP)
Others
Publication year
2020
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Neurotherapeutics
ISSN
1933-7213
e-ISSN
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Volume of the periodical
17
Issue of the periodical within the volume
1
Country of publishing house
US - UNITED STATES
Number of pages
11
Pages from-to
329-339
UT code for WoS article
000511657800028
EID of the result in the Scopus database
2-s2.0-85076739582