Concentrations of pro-thyrotropin-releasing hormone (pro-TRH) were studied in the brain of the Weaver ataxic mouse, the Purkinje cell degenerative mouse (pcd-ataxic mouse), the Staggerer ataxic mouse and the C3H mouse. The brain tissue was dissected into 7 parts, e.g., hypothalamus, cerebrum, thalamus, striatum, brain stem, cerebellum and cervical spinal cord. Pro-TRH concentrations in each part of the brain were measured by radioimmunoassay. Pro-TRH concentrations in the brain of Weaver ataxic mice, pcd-ataxic mice and Staggerer ataxic mice were significantly higher in the thalamus, brain stem, cerebellum and cervical spinal cord. Pro-TRH concentrations in the hypothalamus, striatum and cerebrum of ataxic mice did not differ from those of controls. The elution profile of acetic acid extracted cerebellum of ataxic mice on Sephadex G-50 was identical to that of synthetic pro-TRH. These findings suggest that changes in pro-TRH concentrations in the brain may play a pathophysiological role in ataxic mice.
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http://dx.doi.org/10.1016/0022-510x(90)90257-n | DOI Listing |
Nat Metab
January 2025
CECAD Excellence Center, University of Cologne, Cologne, Germany.
Dysfunctions in autophagy, a cellular mechanism for breaking down components within lysosomes, often lead to neurodegeneration. The specific mechanisms underlying neuronal vulnerability due to autophagy dysfunction remain elusive. Here we show that autophagy contributes to cerebellar Purkinje cell (PC) survival by safeguarding their glycolytic activity.
View Article and Find Full Text PDFPLoS One
December 2024
Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, OK, United States of America.
Here, we describe a spontaneous mouse mutant with a deletion in a predicted gene 2310061I04Rik (Rik) of unknown function located on chromosome 17. A 59 base pair long deletion occurred in the first intron of the Rik gene and disrupted its expression. Riknull mice were born healthy and appeared anatomically normal up to two weeks of age.
View Article and Find Full Text PDFNeurobiol Dis
October 2024
Centro de Biología Molecular Severo Ochoa (CSIC-UAM), Nicolás Cabrera 1, 28049 Madrid, Spain; Departamento de Biología Molecular, Universidad Autónoma de Madrid, Francisco Tomás y Valiente, 7, Ciudad Universitaria de Cantoblanco, 28049 Madrid, Spain; Instituto de Investigación Sanitaria Puerta de Hierro, Segovia de Arana, Hospital Universitario Puerta de Hierro, Joaquín Rodrigo 1, Majadahonda, 28222 Madrid, Spain. Electronic address:
Friedreich ataxia is a hereditary neurodegenerative disorder resulting from reduced levels of the protein frataxin due to an expanded GAA repeat in the FXN gene. This deficiency causes progressive degeneration of specific neuronal populations in the cerebellum and the consequent loss of movement coordination and equilibrium, which are some of the main symptoms observed in affected individuals. Like in other neurodegenerative diseases, previous studies suggest that glial cells could be involved in the neurodegenerative process and disease progression in patients with Friedreich ataxia.
View Article and Find Full Text PDFBrain Behav Immun
October 2024
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan; Translational Neuroscience Research Center, Graduate School of Medicine, and School of Pharmacy at Fukuoka, International University of Health and Welfare, Fukuoka, Japan; Department of Neurology, Brain and Nerve Center, Fukuoka Central Hospital, International University of Health and Welfare, Fukuoka, Japan. Electronic address:
Multiple system atrophy (MSA) is a severe α-synucleinopathy facilitated by glial reactions; the cerebellar variant (MSA-C) preferentially involves olivopontocerebellar fibres with conspicuous demyelination. A lack of aggressive models that preferentially involve olivopontocerebellar tracts in adulthood has hindered our understanding of the mechanisms of demyelination and neuroaxonal loss, and thus the development of effective treatments for MSA. We therefore aimed to develop a rapidly progressive mouse model that recaptures MSA-C pathology.
View Article and Find Full Text PDFMol Ther
July 2024
Université Paris Cité, Inserm, Maladies neurodéveloppementales et neurovasculaires, F-75019 Paris, France. Electronic address:
Neuroglobin, a member of the globin superfamily, is abundant in the brain, retina, and cerebellum of mammals and localizes to mitochondria. The protein exhibits neuroprotective capacities by participating in electron transfer, oxygen supply, and protecting against oxidative stress. Our objective was to determine whether neuroglobin overexpression can be used to treat neurological disorders.
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