Patterned cerebellar Purkinje cell death in a transgenic mouse model of Niemann Pick type A/B disease

2001 ◽  
Vol 13 (10) ◽  
pp. 1873-1880 ◽  
Author(s):  
Justyna Sarna ◽  
Silvia R. P. Miranda ◽  
Edward H. Schuchman ◽  
Richard Hawkes
Keyword(s):  
Cell Death ◽  
Mouse Model ◽  
Purkinje Cell ◽  
Transgenic Mouse ◽  
Type A ◽  
Transgenic Mouse Model ◽  
Cerebellar Purkinje Cell ◽  
Purkinje Cell Death ◽  
Niemann Pick

scholarly journals Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease

2015 ◽  
Vol 7 (276) ◽  
pp. 276ra26-276ra26 ◽  
Author(s):  
Charles H. Vite ◽  
Jessica H. Bagel ◽  
Gary P. Swain ◽  
Maria Prociuk ◽  
Tracey U. Sikora ◽  
...  
Keyword(s):  
Cell Death ◽  
Purkinje Cell ◽  
Cerebellar Dysfunction ◽  
Purkinje Cell Death ◽  
Niemann Pick

scholarly journals In Vivo Analysis of the Climbing Fiber-Purkinje Cell Circuit in SCA2-58Q Transgenic Mouse Model

2018 ◽  
Vol 17 (5) ◽  
pp. 590-600 ◽  
Author(s):  
Polina A. Egorova ◽  
Alexandra V. Gavrilova ◽  
Ilya B. Bezprozvanny
Keyword(s):  
Mouse Model ◽  
Purkinje Cell ◽  
Transgenic Mouse ◽  
Transgenic Mouse Model ◽  
Climbing Fiber ◽  
In Vivo Analysis ◽  
Cell Circuit

scholarly journals PAX6 Expression and Retinal Cell Death in a Transgenic Mouse Model for Acute Angle-Closure Glaucoma

2015 ◽  
Vol 24 (6) ◽  
pp. 426-432
Author(s):  
Dinu Stanescu-Segall ◽  
Kerstin Birke ◽  
Andreas Wenzel ◽  
Christian Grimm ◽  
Sorguel Orgul ◽  
...  
Keyword(s):  
Cell Death ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Transgenic Mouse Model ◽  
Acute Angle ◽  
Retinal Cell ◽  
Angle Closure ◽  
Pax6 Expression ◽  
Acute Angle Closure ◽  
Acute Angle Closure Glaucoma

Celastrol Blocks Neuronal Cell Death and Extends Life in Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

2005 ◽  
Vol 2 (5) ◽  
pp. 246-254 ◽  
Author(s):  
Mahmoud Kiaei ◽  
Khatuna Kipiani ◽  
Susanne Petri ◽  
Junyu Chen ◽  
Noel Y. Calingasan ◽  
...  
Keyword(s):  
Cell Death ◽  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Neuronal Cell Death ◽  
Neuronal Cell ◽  
Transgenic Mouse Model ◽  
Lateral Sclerosis

Melatonin alleviates asphyxial cardiac arrest-induced cerebellar Purkinje cell death by attenuation of oxidative stress

2019 ◽  
Vol 320 ◽  
pp. 112983 ◽  
Author(s):  
Jeong Hwi Cho ◽  
Hyun-Jin Tae ◽  
In-Shik Kim ◽  
Minah Song ◽  
Hyunjung Kim ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Cell Death ◽  
Cardiac Arrest ◽  
Purkinje Cell ◽  
Cerebellar Purkinje Cell ◽  
Purkinje Cell Death ◽  
Asphyxial Cardiac Arrest

scholarly journals From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington'sdisease

1999 ◽  
Vol 354 (1386) ◽  
pp. 971-979 ◽  
Author(s):  
Stephen W. Davies ◽  
Turmaine Mark ◽  
Barbara A. Cozens ◽  
Aysha S. Raza ◽  
Amarbirpal Mahal ◽  
...  
Keyword(s):  
Cell Death ◽  
Mouse Model ◽  
Transgenic Mouse ◽  
Neuronal Cell Death ◽  
Neuronal Cell ◽  
Transgenic Mouse Model ◽  
Repeat Sequence ◽  
Intranuclear Inclusions ◽  
Full Characterization ◽  
Exon 1

Huntington'sdisease (HD) is an inherited progressive neurodegenerative disease caused by the expansion of a polyglutamine repeat sequence within a novel protein. Recent work has shown that abnormal intranuclear inclusions of aggregated mutant protein within neurons is a characteristic feature shared by HD and several other diseases involving glutamine repeat expansion. This suggests that in each of the these disorders the affected nerve cells degenerate as a result of these abnormal inclusions. A transgenic mouse model of HD has been generated by introducing exon 1 of the HD gene containing a highly expanded CAG sequence into the mouse germline. These mice develop widespread neuronal intranuclear inclusions and neurodegeneration specifically within those areas of the brain known to degenerate in HD. We have investigated the sequence of pathological changes that occur after the formation of nuclear inclusions and that precede neuronal cell death in these cells. Although the relation between inclusion formation and neurodegeneration has recently been questioned, a full characterization of the pathways linking protein aggregation and cell death will resolve some of these controversies and will additionally provide new targets for potential therapies.

Sign in / Sign up

Export Citation Format

Share Document