scholarly journals Region-specific Dissociation of Neuronal Loss and Neurofibrillary Pathology in a Mouse Model of Tauopathy

2006 ◽  
Vol 168 (5) ◽  
pp. 1598-1607 ◽  
Author(s):  
Tara L. Spires ◽  
Jennifer D. Orne ◽  
Karen SantaCruz ◽  
Rose Pitstick ◽  
George A. Carlson ◽  
...  
Keyword(s):  
Mouse Model ◽  
Neuronal Loss ◽  
Neurofibrillary Pathology

scholarly journals Acceleration and persistence of neurofibrillary pathology in a mouse model of tauopathy following anesthesia

2009 ◽  
Vol 23 (8) ◽  
pp. 2595-2604 ◽  
Author(s):  
Emmanuel Planel ◽  
Alexis Bretteville ◽  
Li Liu ◽  
Laszlo Virag ◽  
Angela L. Du ◽  
...  
Keyword(s):  
Mouse Model ◽  
Neurofibrillary Pathology

scholarly journals Bowman–Birk inhibitor suppresses autoimmune inflammation and neuronal loss in a mouse model of multiple sclerosis

2008 ◽  
Vol 271 (1-2) ◽  
pp. 191-202 ◽  
Author(s):  
Tarik Touil ◽  
Bogoljub Ciric ◽  
Elvira Ventura ◽  
Kenneth S. Shindler ◽  
Bruno Gran ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Mouse Model ◽  
Neuronal Loss ◽  
Autoimmune Inflammation

scholarly journals Overexpression of UBQLN1 reduces neuropathology in the P497S UBQLN2 mouse model of ALS/FTD

2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Shaoteng Wang ◽  
Micaela Tatman ◽  
Mervyn J. Monteiro
Keyword(s):  
Mouse Model ◽  
Neuronal Loss ◽  
Body Weight Loss ◽  
Missense Mutations ◽  
Homologous Proteins ◽  
Female Mice ◽  
Beneficial Effects ◽  
Therapeutic Benefits ◽  
Gender Specific ◽  
Lateral Sclerosis

Abstract Missense mutations in UBQLN2 cause X-linked dominant inheritance of amyotrophic lateral sclerosis with frontotemporal dementia (ALS/FTD). UBQLN2 belongs to a family of four highly homologous proteins expressed in humans that play diverse roles in maintaining proteostasis, but whether one isoform can substitute for another is not known. Here, we tested whether overexpression of UBQLN1 can alleviate disease in the P497S UBQLN2 mouse model of ALS/FTD by crossing transgenic (Tg) mouse lines expressing the two proteins and characterizing the resulting genotypes using a battery of pathologic and behavioral tests. The pathologic findings revealed UBQLN1 overexpression dramatically reduced the burden of UBQLN2 inclusions, neuronal loss and disturbances in proteostasis in double Tg mice compared to single P497S Tg mice. The beneficial effects of UBQLN1 overexpression were primarily confirmed by behavioral improvements seen in rotarod performance and grip strength in male, but not female mice. Paradoxically, although UBQLN1 overexpression reduced pathologic signatures of disease in P497S Tg mice, female mice had larger percentage of body weight loss than males, and this correlated with a corresponding lack of behavioral improvements in the females. These findings lead us to speculate that methods to upregulate UBQLN1 expression may reduce pathogenicity caused by UBQLN2 mutations, but may also lead to gender-specific outcomes that will have to be carefully weighed with the therapeutic benefits of UBQLN1 upregulation.

scholarly journals 7,8-Dihydroxyflavone, a Small Molecule TrkB Agonist, Improves Spatial Memory and Increases Thin Spine Density in a Mouse Model of Alzheimer Disease-Like Neuronal Loss

PLoS ONE ◽  
2014 ◽  
Vol 9 (3) ◽  
pp. e91453 ◽  
Author(s):  
Nicholas A. Castello ◽  
Michael H. Nguyen ◽  
Jenny D. Tran ◽  
David Cheng ◽  
Kim N. Green ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Mouse Model ◽  
Spatial Memory ◽  
Small Molecule ◽  
Neuronal Loss ◽  
Spine Density
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