Cell cycle activation in striatal neurons from Huntington's disease patients and rats treated with 3‐nitropropionic acid

2008 ◽  
Vol 26 (7) ◽  
pp. 665-671 ◽  
Author(s):  
Carme Pelegrí ◽  
Joaquim Duran‐Vilaregut ◽  
Jaume Valle ◽  
Natàlia Crespo‐Biel ◽  
Isidre Ferrer ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Striatal Neurons ◽  
Nitropropionic Acid ◽  
Cell Cycle Activation ◽  
3 Nitropropionic Acid

scholarly journals Exaggerated mitophagy: a weapon of striatal destruction in the brain?

2020 ◽  
Vol 48 (2) ◽  
pp. 709-717
Author(s):  
Srinivasa Subramaniam
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Mutant Huntingtin ◽  
Striatal Neurons ◽  
Mitochondrial Complex ◽  
Complex Ii ◽  
Nitropropionic Acid ◽  
Striatal Lesion ◽  
The Brain ◽  
3 Nitropropionic Acid

Mechanisms responsible for neuronal vulnerability in the brain remain unclear. Striatal neurons are preferentially damaged by 3-nitropropionic acid (3-NP), a mitochondrial complex-II inhibitor, causing striatal damage reminiscent of Huntington's disease (HD), but the mechanisms of the selectivity are not as well understood. We have discovered that Rhes, a protein enriched in the striatum, removes mitochondria via the mitophagy process. The process becomes intensified in the presence of 3-NP, thereby eliminating most of the mitochondria from the striatum. We put forward the hypothesis that Rhes acts as a ‘mitophagy ligand' in the brain and promotes mitophagy via NIX, a mitophagy receptor. Since Rhes interacts and promotes toxicity in association with mutant huntingtin (mHTT), the genetic cause of HD, it is tempting to speculate on whether the exaggerated mitophagy may be a contributing factor to the striatal lesion found in HD. Thus, Rhes-mediated exaggerated mitophagy may act as a weapon of striatal destruction in the brain.

Neurturin gene therapy improves motor function and prevents death of striatal neurons in a 3-nitropropionic acid rat model of Huntington's disease

2007 ◽  
Vol 26 (2) ◽  
pp. 375-384 ◽  
Author(s):  
Shilpa Ramaswamy ◽  
Jodi L. McBride ◽  
Christopher D. Herzog ◽  
Eugene Brandon ◽  
Mehdi Gasmi ◽  
...  
Keyword(s):  
Gene Therapy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Motor Function ◽  
Rat Model ◽  
Striatal Neurons ◽  
Nitropropionic Acid ◽  
3 Nitropropionic Acid

Neuroprotective activity of tetramethylpyrazine against 3-nitropropionic acid induced Huntington’s disease-like symptoms in rats

2018 ◽  
Vol 105 ◽  
pp. 1254-1268 ◽  
Author(s):  
Ravi Chandra Sekhara Reddy Danduga ◽  
Subba Reddy Dondapati ◽  
Phani Kumar Kola ◽  
Lilly Grace ◽  
Rahil Vandana Bisky Tadigiri ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuroprotective Activity ◽  
Nitropropionic Acid ◽  
3 Nitropropionic Acid

scholarly journals Chronic 3-Nitropropionic Acid Treatment in Baboons Replicates the Cognitive and Motor Deficits of Huntington’s Disease

1996 ◽  
Vol 16 (9) ◽  
pp. 3019-3025 ◽  
Author(s):  
Stéphane Palfi ◽  
Robert J. Ferrante ◽  
Emmanuel Brouillet ◽  
M. Flint Beal ◽  
Robert Dolan ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Acid Treatment ◽  
Motor Deficits ◽  
Nitropropionic Acid ◽  
3 Nitropropionic Acid
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