NAD-biosynthetic enzyme NMNAT1 reduces early behavioral abnormalities in the htau mouse model of tauopathy

2015 ◽  
Vol 68 ◽  
pp. 96
Author(s):  
Francesca Rossi ◽  
Weina Meng ◽  
Philippine C. Geiszler ◽  
Malcolm Prior ◽  
Anna Herd-Smith ◽  
...  
2020 ◽  
Vol 103 ◽  
pp. 106842
Author(s):  
Dilara Bahceci ◽  
Lyndsey Leigh Anderson ◽  
Cassandra Veronica Occelli Hanbury Brown ◽  
Cilla Zhou ◽  
Jonathon Carl Arnold

2010 ◽  
Vol 74 (11) ◽  
pp. 2299-2306 ◽  
Author(s):  
Nakaba MURATA ◽  
Kazuma MURAKAMI ◽  
Yusuke OZAWA ◽  
Noriaki KINOSHITA ◽  
Kazuhiro IRIE ◽  
...  

Author(s):  
Patricia Cogram ◽  
Robert M. J. Deacon ◽  
Jennifer L. Warner-Schmidt ◽  
Melanie J. von Schimmelmann ◽  
Brett S. Abrahams ◽  
...  

1994 ◽  
Vol 9 (3) ◽  
pp. 234-241 ◽  
Author(s):  
Barry E. Kosofsky ◽  
Aaron S. Wilkins ◽  
Pierre Gressens ◽  
Philippe Evrard

2016 ◽  
Vol 10 (4) ◽  
pp. e0004674 ◽  
Author(s):  
Jianchun Xiao ◽  
Ye Li ◽  
Emese Prandovszky ◽  
Geetha Kannan ◽  
Raphael P. Viscidi ◽  
...  

2015 ◽  
Vol 24 (21) ◽  
pp. 6198-6212 ◽  
Author(s):  
Casey Cook ◽  
Silvia S. Kang ◽  
Yari Carlomagno ◽  
Wen-Lang Lin ◽  
Mei Yue ◽  
...  

2020 ◽  
Author(s):  
Tyler B. Johnson ◽  
Katherine A. White ◽  
Jacob T. Cain ◽  
Logan Langin ◽  
Melissa A. Pratt ◽  
...  

AbstractCLN8 disease is a rare form of neuronal ceroid lipofuscinosis caused by biallelic mutations in the CLN8 gene, which encodes a transmembrane endoplasmic reticulum protein involved in trafficking of lysosomal enzymes. CLN8 disease patients present with myoclonus, tonic-clonic seizures, and progressive declines in cognitive and motor function, with many cases resulting in premature death early in life. There are currently no treatments that can cure the disease or substantially slow disease progression. Using a mouse model of CLN8 disease, we tested the safety and efficacy of an intracerebroventricularly (ICV)-delivered self-complementary AAV9 (scAAV9) gene therapy vector driving expression of human CLN8. A single neonatal injection was safe and well-tolerated, resulting in robust transgene expression throughout the brain and spinal cord from 4 to 24 months, reducing histopathological and behavioral hallmarks of the disease and completely restoring lifespan from 10 months in untreated animals to beyond 24 months of age in treated animals. These results demonstrate, by far, the most successful rescue reported in an animal model of CLN8 disease, and supports gene therapy as a promising therapeutic strategy for this disorder.


Author(s):  
Yuta Hara ◽  
Yukio Ago ◽  
Momoko Higuchi ◽  
Shigeru Hasebe ◽  
Takanobu Nakazawa ◽  
...  

Nutrients ◽  
2020 ◽  
Vol 12 (2) ◽  
pp. 445 ◽  
Author(s):  
Mariya A. Borisova ◽  
Olga A. Snytnikova ◽  
Ekaterina A. Litvinova ◽  
Kseniya M. Achasova ◽  
Tatiana I. Babochkina ◽  
...  

Growing evidence suggests that intestinal mucosa homeostasis impacts immunity, metabolism, the Central Nervous System (CNS), and behavior. Here, we investigated the effect of the monosaccharide fucose on inflammation, metabolism, intestinal microbiota, and social behavior in the Dextran Sulfate Sodium (DSS)-induced chronic colitis mouse model. Our data show that chronic colitis is accompanied by the decrease of the serum tryptophan level and the depletion of the intestinal microbiota, specifically tryptophan-producing E. coli and Bifidobacterium. These changes are associated with defects in the male mouse social behavior such as a lack of preference towards female bedding in an odor preference test. The addition of fucose to the test animals’ diet altered the bacterial community, increased the abundance of tryptophan-producing E. coli, normalized blood tryptophan levels, and ameliorated social behavior deficits. At the same time, we observed no ameliorating effect of fucose on colon morphology and colitis. Our results suggest a possible mechanism by which intestinal inflammation affects social behavior in male mice. We propose fucose as a promising prebiotic, since it creates a favorable environment for the beneficial bacteria that promote normalization of serum tryptophan level and amelioration of the behavioral abnormalities in the odor preference test.


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