scholarly journals Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis

Brain ◽  
2013 ◽  
Vol 136 (12) ◽  
pp. 3766-3774 ◽  
Author(s):  
Fenfen Wu ◽  
Wentao Mi ◽  
Stephen C. Cannon
Keyword(s):  
Mouse Model ◽  
Periodic Paralysis ◽  
Beneficial Effects ◽  
Hypokalaemic Periodic Paralysis

Beneficial Effects of Fingolimod on Social Interaction, CNS and Peripheral Immune Response in the BTBR Mouse Model of Autism

Neuroscience ◽  
2020 ◽  
Vol 435 ◽  
pp. 22-32
Author(s):  
Roberta De Simone ◽  
Alessia Butera ◽  
Monica Armida ◽  
Antonella Pezzola ◽  
Monica Boirivant ◽  
...  
Keyword(s):  
Immune Response ◽  
Social Interaction ◽  
Mouse Model ◽  
Beneficial Effects ◽  
Btbr Mouse ◽  
Peripheral Immune Response

scholarly journals Beneficial Effects of Acetyl-DL-Leucine (ADLL) in a Mouse Model of Sandhoff Disease

2020 ◽  
Vol 9 (4) ◽  
pp. 1050 ◽  
Author(s):  
Ecem Kaya ◽  
David A. Smith ◽  
Claire Smith ◽  
Barry Boland ◽  
Michael Strupp ◽  
...  
Keyword(s):  
Mouse Model ◽  
Sandhoff Disease ◽  
Lysosomal Storage ◽  
Gm2 Ganglioside ◽  
Beneficial Effects ◽  
New Therapeutic Approaches ◽  
Late Endosomes ◽  
Altered Glucose ◽  
Cerebellar Ataxias ◽  
Niemann Pick

Sandhoff disease is a rare neurodegenerative lysosomal storage disease associated with the storage of GM2 ganglioside in late endosomes/lysosomes. Here, we explored the efficacy of acetyl-DL-leucine (ADLL), which has been shown to improve ataxia in observational studies in patients with Niemann–Pick Type C1 and other cerebellar ataxias. We treated a mouse model of Sandhoff disease (Hexb-/-) (0.1 g/kg/day) from 3 weeks of age with this orally available drug. ADLL produced a modest but significant increase in life span, accompanied by improved motor function and reduced glycosphingolipid (GSL) storage in the forebrain and cerebellum, in particular GA2. ADLL was also found to normalize altered glucose and glutamate metabolism, as well as increasing autophagy and the reactive oxygen species (ROS) scavenger, superoxide dismutase (SOD1). Our findings provide new insights into metabolic abnormalities in Sandhoff disease, which could be targeted with new therapeutic approaches, including ADLL.

scholarly journals Hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism: an uncommon cause of acute muscle paralysis

2021 ◽  
Vol 14 (6) ◽  
pp. e240666
Author(s):  
Alvin Oliver Payus ◽  
Sat Lin Liew ◽  
Nee Tiong ◽  
Norlaila Mustafa
Keyword(s):  
Lower Limb ◽  
Beta Blockers ◽  
Thyroid Function Test ◽  
Periodic Paralysis ◽  
Subclinical Hyperthyroidism ◽  
Thyroid Medication ◽  
Lower Limb Paralysis ◽  
Clinical Phenomenon ◽  
Potassium Replacement ◽  
Hypokalaemic Periodic Paralysis

Hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism is an uncommon clinical phenomenon characterised by lower limb paralysis secondary to hypokalaemia in the background of subclinical hyperthyroidism. In this article, we report a patient who presented with progressive lower limb muscle weakness secondary to hypokalaemia that was refractory to potassium replacement therapy. He has no diarrhoea, no reduced appetite and was not taking any medication that can cause potassium wasting. Although he was clinically euthyroid, his thyroid function test revealed subclinical hyperthyroidism. His 24-hour urine potassium level was normal, which makes a rapid transcellular shift of potassium secondary to subclinical hyperthyroidism as the possible cause. He was successfully treated with potassium supplements, non-selective beta-blockers and anti-thyroid medication. This case report aimed to share an uncommon case of hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism, which to our knowledge, only a few has been reported in the literature.

scholarly journals Berberine Prevents Disease Progression of Nonalcoholic Steatohepatitis through Modulating Multiple Pathways

Cells ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 210
Author(s):  
Yanyan Wang ◽  
Yun-Ling Tai ◽  
Derrick Zhao ◽  
Yuan Zhang ◽  
Junkai Yan ◽  
...  
Keyword(s):  
Bile Acid ◽  
Mouse Model ◽  
Disease Progression ◽  
Nonalcoholic Steatohepatitis ◽  
Noncoding Rna ◽  
Metabolic Diseases ◽  
Immune Cell ◽  
Stellate Cell ◽  
Clinical Investigation ◽  
Beneficial Effects

Background and Aims: The disease progression of nonalcoholic fatty liver disease (NAFLD) from simple steatosis (NAFL) to nonalcoholic steatohepatitis (NASH) is driven by multiple factors. Berberine (BBR) is an ancient Chinese medicine and has various beneficial effects on metabolic diseases, including NAFLD/NASH. However, the underlying mechanisms remain incompletely understood due to the limitation of the NASH animal models used. Methods: A high-fat and high-fructose diet-induced mouse model of NAFLD, the best available preclinical NASH mouse model, was used. RNAseq, histological, and metabolic pathway analyses were used to identify the potential signaling pathways modulated by BBR. LC–MS was used to measure bile acid levels in the serum and liver. The real-time RT-PCR and Western blot analysis were used to validate the RNAseq data. Results: BBR not only significantly reduced hepatic lipid accumulation by modulating fatty acid synthesis and metabolism but also restored the bile acid homeostasis by targeting multiple pathways. In addition, BBR markedly inhibited inflammation by reducing immune cell infiltration and inhibition of neutrophil activation and inflammatory gene expression. Furthermore, BBR was able to inhibit hepatic fibrosis by modulating the expression of multiple genes involved in hepatic stellate cell activation and cholangiocyte proliferation. Consistent with our previous findings, BBR’s beneficial effects are linked with the downregulation of microRNA34a and long noncoding RNA H19, which are two important players in promoting NASH progression and liver fibrosis. Conclusion: BBR is a promising therapeutic agent for NASH by targeting multiple pathways. These results provide a strong foundation for a future clinical investigation.

scholarly journals Beneficial Effects of Ramipril Treatment on Diastolic Function, Structural Remodelling and Apoptosis in a Mouse Model of Type 2 Diabetes

2011 ◽  
Vol 20 ◽  
pp. S45-S46
Author(s):  
K. Huynh ◽  
H. Kiriazis ◽  
X. Du ◽  
J. Love ◽  
K. Jandeleit-Dahm ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Mouse Model ◽  
Diastolic Function ◽  
Beneficial Effects
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