Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knockout mice

Loss‐of‐function mutations in progranulin (GRN) are associated with frontotemporal lobar degeneration with intraneuronal ubiquitinated protein accumulations composed primarily of hyperphosphorylated TDP‐43 (FTLD‐TDP). The mechanism by which GRN deficiency causes TDP‐43 pathology or neurodegeneration...

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Veröffentlicht in:	The Journal of pathology 2012-09, Vol.228 (1), p.67-76
Hauptverfasser:	Wils, Hans, Kleinberger, Gernot, Pereson, Sandra, Janssens, Jonathan, Capell, Anja, Van Dam, Debby, Cuijt, Ivy, Joris, Geert, De Deyn, Peter P, Haass, Christian, Van Broeckhoven, Christine, Kumar-Singh, Samir
Format:	Artikel
Sprache:	eng
Schlagworte:	ageing Animals Behavior, Animal - physiology Biological and medical sciences Brain - metabolism Brain - pathology Cellular Senescence DNA-Binding Proteins - genetics DNA-Binding Proteins - metabolism Female Frontotemporal Lobar Degeneration - genetics Frontotemporal Lobar Degeneration - mortality Frontotemporal Lobar Degeneration - pathology FTLD Gene Expression Gliosis - metabolism Gliosis - pathology IGF-1 Intercellular Signaling Peptides and Proteins - physiology Investigative techniques, diagnostic techniques (general aspects) Liver - pathology Longevity - physiology Male Medical sciences Mice Mice, Knockout mouse model Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques Phosphorylation progranulin Survival Rate TDP-43 Ubiquitin - metabolism
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