The neuropathology and clinical phenotype of FTD with progranulin mutations
IRA Mackenzie - Acta neuropathologica, 2007 - Springer
… -U [31] and we later proposed that they are a pathological marker for familial FTD caused
by a specific gene defect [20]. Although lentiform NII are an absolutely sensitive marker for …
by a specific gene defect [20]. Although lentiform NII are an absolutely sensitive marker for …
Progranulin deficiency promotes persistent neuroinflammation and causes regional pathology in the hippocampus following traumatic brain injury
X Zheng, T Mi, R Wang, Z Zhang, W Li, J Zhao, P Yang… - Glia, 2022 - Wiley Online Library
… This study aimed to determine whether progranulin (PGRN), a major modulator of microglial
activity, plays a role in the progressive damage following TBI. PGRN-deficient and wild-type …
activity, plays a role in the progressive damage following TBI. PGRN-deficient and wild-type …
Losing protein in the brain: the case of progranulin
R Ghidoni, A Paterlini, V Albertini, G Binetti, L Benussi - Brain research, 2012 - Elsevier
… Since the discovery of progranulin mutations there has been an interest in developing
mouse models of progranulin deficiency with the expectation that aspects of the pathological …
mouse models of progranulin deficiency with the expectation that aspects of the pathological …
Pathological correlates of white matter hyperintensities in a case of progranulin mutation associated frontotemporal dementia
IOC Woollacott, M Bocchetta, CH Sudre, BH Ridha… - Neurocase, 2018 - Taylor & Francis
… ) due to progranulin (GRN) mutations, but their pathological … , then selected five different
brain regions directly matched to … We used immunohistochemistry to assess vascular pathology…
brain regions directly matched to … We used immunohistochemistry to assess vascular pathology…
Corticobasal Syndrome Associated With the A9D Progranulin Mutation
S Spina, JR Murrell, ED Huey… - … of Neuropathology & …, 2007 - academic.oup.com
… pathologic substrate of corticobasal syndrome has been further expanded to include cases
with pathologic … 43)-positive inclusions associated with progranulin (PGRN) mutations. We …
with pathologic … 43)-positive inclusions associated with progranulin (PGRN) mutations. We …
Clinical, genetic, and pathologic characteristics of patients with frontotemporal dementia and progranulin mutations
VM Van Deerlin, EMC Wood, P Moore… - Archives of …, 2007 - jamanetwork.com
… We do not find a deficit for working memory in this series. The detailed description of the
2 cases with a GRN mutation are said to have normal memory. We find a relative deficit for …
2 cases with a GRN mutation are said to have normal memory. We find a relative deficit for …
Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice
Y Tanaka, JK Chambers, T Matsuwaki… - Acta neuropathologica …, 2014 - Springer
… We characterized the brain pathology related to NCL in aged PGRN-deficient mice in the
present study. PGRN deficiency resulted in neuronal loss, increased gliosis, lipofuscin …
present study. PGRN deficiency resulted in neuronal loss, increased gliosis, lipofuscin …
Disease and region specificity of granulin immunopositivities in Alzheimer disease and frontotemporal lobar degeneration
Q Mao, D Wang, Y Li, M Kohler, J Wilson… - … of Neuropathology & …, 2017 - academic.oup.com
… of progranulin message upstream of the Grn D region, … deficient immunoprotection in this
region. These changes might be related to the development of HS and/or TDP-43 pathology in …
region. These changes might be related to the development of HS and/or TDP-43 pathology in …
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis
… Heterozygous loss-of-function mutations in the progranulin (… Recently, it has been shown
that a complete GRN deficiency … a risk factor for FTLD-TDP pathology. In line with a potential …
that a complete GRN deficiency … a risk factor for FTLD-TDP pathology. In line with a potential …
Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis
… lead to progranulin … progranulin-deficient FTD and NCL remains unexplored. Now, Ward
et al. show that patients with heterozygous GRN mutations exhibit clinical and pathological …
et al. show that patients with heterozygous GRN mutations exhibit clinical and pathological …
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