PRKRA ‐Related Disorders: Bilateral Striatal Degeneration in Addition to DYT16 Spectrum

2021 ◽  
Author(s):  
Silvia Masnada ◽  
Diego Martinelli ◽  
Marta Correa‐Vela ◽  
Emanuele Agolini ◽  
Heidy Baide‐Mairena ◽  
...  
Keyword(s):  
Striatal Degeneration

Eye movement deficits in X-linked dystonia-parkinsonism are related to striatal degeneration

2019 ◽  
Vol 61 ◽  
pp. 170-178 ◽  
Author(s):  
Andreas Sprenger ◽  
Henrike Hanssen ◽  
Imke Hagedorn ◽  
Jannik Prasuhn ◽  
Raymond L. Rosales ◽  
...  
Keyword(s):  
Eye Movement ◽  
Striatal Degeneration

Early inhibition of TNFα increases 6-hydroxydopamine-induced striatal degeneration

2007 ◽  
Vol 1147 ◽  
pp. 240-247 ◽  
Author(s):  
Carmelina Gemma ◽  
Briony Catlow ◽  
Michael Cole ◽  
Charles Hudson ◽  
Amy Samec ◽  
...  
Keyword(s):  
Striatal Degeneration ◽  
Early Inhibition ◽  
6 Hydroxydopamine

scholarly journals Transplantation of Adipose-Derived Stem Cells Alleviates Striatal Degeneration in a Transgenic Mouse Model for Multiple System Atrophy

2020 ◽  
Vol 29 ◽  
pp. 096368972096018
Author(s):  
Christine Chang ◽  
Jen-Wei Liu ◽  
Bo Cheng Chen ◽  
Zhe Sheng Jiang ◽  
Chi Tang Tu ◽  
...  
Keyword(s):  
Mouse Model ◽  
Transgenic Mice ◽  
Multiple System Atrophy ◽  
Transgenic Mouse ◽  
Neurodegenerative Disorder ◽  
Ex Vivo ◽  
Transgenic Mouse Model ◽  
Intracerebral Injection ◽  
Nigrostriatal Pathway ◽  
Striatal Degeneration

Patients with multiple system atrophy (MSA), a progressive neurodegenerative disorder of adult onset, were found less than 9 years of life expectancy after onset. The disorders include bradykinesia and rigidity commonly seen in Parkinsonism disease and additional signs such as autonomic dysfunction, ataxia, or dementia. In clinical treatments, MSA poorly responds to levodopa, the drug used to remedy Parkinsonism disease. The exact cause of MSA is still unknown, and exploring a therapeutic solution to MSA remains critical. A transgenic mouse model was established to study the feasibility of human adipose-derived stem cell (ADSC) therapy in vivo. The human ADSCs were transplanted into the striatum of transgenic mice via intracerebral injection. As compared with sham control, we reported significantly enhanced rotarod performance of transgenic mice treated with ADSC at an effective dose, 2 × 105 ADSCs/mouse. Our ex vivo feasibility study supported that intracerebral transplantation of ADSC might alleviate striatal degeneration in MSA transgenic mouse model by improving the nigrostriatal pathway for dopamine, activating autophagy for α-synuclein clearance, decreasing inflammatory signal, and further cell apoptosis, improving myelination and cell survival at caudate-putamen.

scholarly journals Calpain Is a Major Cell Death Effector in Selective Striatal Degeneration InducedIn Vivoby 3-Nitropropionate: Implications for Huntington's Disease

2003 ◽  
Vol 23 (12) ◽  
pp. 5020-5030 ◽  
Author(s):  
Nicolas Bizat ◽  
Jean-Michel Hermel ◽  
Frédéric Boyer ◽  
Carine Jacquard ◽  
Christophe Créminon ◽  
...  
Keyword(s):  
Cell Death ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Striatal Degeneration ◽  
Death Effector

Riluzole Reduces Incidence of Abnormal Movements but Not Striatal Cell Death in a Primate Model of Progressive Striatal Degeneration

1997 ◽  
Vol 146 (1) ◽  
pp. 135-141 ◽  
Author(s):  
Stéphane Palfi ◽  
Danielle Riche ◽  
Emmanuel Brouillet ◽  
Marie-Caroline Guyot ◽  
Véronique Mary ◽  
...  
Keyword(s):  
Cell Death ◽  
Primate Model ◽  
Abnormal Movements ◽  
Striatal Degeneration

Spontaneous Striatal Degeneration in a Monkey

1944 ◽  
Vol 3 (1) ◽  
pp. 49-62 ◽  
Author(s):  
Richard Richter ◽  
Heinrich Klüver
Keyword(s):  
Striatal Degeneration

scholarly journals Loss of Hap1 selectively promotes striatal degeneration in Huntington disease mice

2020 ◽  
Vol 117 (33) ◽  
pp. 20265-20273 ◽  
Author(s):  
Qiong Liu ◽  
Siying Cheng ◽  
Huiming Yang ◽  
Louyin Zhu ◽  
Yongcheng Pan ◽  
...  
Keyword(s):  
Huntington Disease ◽  
Cellular Stress ◽  
Neuronal Loss ◽  
Ideal Model ◽  
Adeno Associated Virus ◽  
Multiple Factors ◽  
Striatal Degeneration ◽  
Mouse Striatum

Huntington disease (HD) is an ideal model for investigating selective neurodegeneration, as expanded polyQ repeats in the ubiquitously expressed huntingtin (HTT) cause the preferential neurodegeneration in the striatum of the HD patient brains. Here we report that adeno-associated virus (AAV) transduction-mediated depletion of Hap1, the first identified huntingtin-associated protein, in adult HD knock-in (KI) mouse brains leads to selective neuronal loss in the striatum. Further, Hap1 depletion-mediated neuronal loss via AAV transduction requires the presence of mutant HTT. Rhes, a GTPase that is enriched in the striatum and sumoylates mutant HTT to mediate neurotoxicity, binds more N-terminal HTT when Hap1 is deficient. Consistently, more soluble and sumoylated N-terminal HTT is presented in HD KI mouse striatum when HAP1 is absent. Our findings suggest that both Rhes and Hap1 as well as cellular stress contribute to the preferential neurodegeneration in HD, highlighting the involvement of multiple factors in selective neurodegeneration.

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