During nervous system development, neural progenitors arise in proliferative zones, then exit the cell cycle and differentiate as they migrate away from these zones. The neuronal protein BM88/Cend1 has been implicated in coordination of cell cycle exit and differentiation of neuronal precursors. To further elucidate its function we generated Cend1 knock-out mice and analyzed their phenotype during postnatal cerebellar development. Cend1(-/-) mice showed no overt abnormalities in the gross anatomy of the cerebellum or other brain regions. However, detailed analysis revealed alterations in cerebellar layering arising from increased proliferation of granule cell precursors, delayed radial granule cell migration and impaired Purkinje cell differentiation. Accordingly, expression of Patched1, cyclin D1, reelin and brain-derived neurotrophic factor, which correlate with morphological development of the cerebellum, was altered in Cend1(-/-) mice. The observed anatomical and molecular alterations were accompanied by deficits in motor behaviour. Our results suggest that Cend1 is required for normal cerebellar development.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.mcn.2010.01.011 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Background: There are no disease modifying therapies for Huntington's disease (HD), a rare but fatal genetic neurodegenerative condition. To develop and test new management strategies, a better understanding of the mechanisms underlying HD progression is needed. Aberrant changes in thalamo-cortical and striato-cerebellar circuitry have been observed in asymptomatic HD, along with transient enlargement of the dentate nucleus.
View Article and Find Full Text PDFEffects of physiotherapy on degenerative cerebellar ataxia: a systematic review and meta-analysis.
Front Neurol
January 2025
Department of Rehabilitation, Maruki Memorial Medical and Social Welfare Center, Saitama, Japan.
Background: Evidence of the effectiveness of physiotherapy, including muscle strength training, coordination training, aerobic exercise, cycling regimen, balance training, gait training, and activity of daily living training, in patients with degenerative cerebellar ataxia (DCA) was insufficient for clinical decision making. We aimed to explore clinical outcomes and examine the parameters associated with physical impairment and activity in people with DCA based on preregistration (PROSPERO: CRD42024493883).
Methods: The PubMed, Cochrane Library, CHINAL, and PEDro databases were searched for relevant randomized controlled trials (RCTs).
Ultrastructural Study and Immunohistochemical Characteristics of Mesencephalic Tegmentum in Juvenile Chum Salmon () Brain After Acute Traumatic Injury.
Int J Mol Sci
January 2025
A.V. Zhirmunsky National Scientific Center of Marine Biology, Far Eastern Branch, Russian Academy of Sciences, 690041 Vladivostok, Russia.
The ultrastructural organization of the nuclei of the tegmental region in juvenile chum salmon () was examined using transmission electron microscopy (TEM). The dorsal tegmental nuclei (DTN), the nucleus of (NFLM), and the nucleus of the oculomotor nerve (NIII) were studied. The ultrastructural examination provided detailed ultrastructural characteristics of neurons forming the tegmental nuclei and showed neuro-glial relationships in them.
View Article and Find Full Text PDFSystematic Analysis of UFMylation Family Genes in Tissues of Mice with Metabolic Dysfunction-Associated Steatotic Liver Disease.
Genes (Basel)
December 2024
Zhejiang Key Laboratory of Medical Epigenetics, Department of Cell Biology and Genetics, School of Basic Medical Sciences, Hangzhou Normal University, Hangzhou 310036, China.
Background/objectives: UFMylation, a newly identified ubiquitin-like modification, modulates a variety of physiological processes, including endoplasmic reticulum homeostasis maintenance, DNA damage response, embryonic development, and tumor progression. Recent reports showed that UFMylation plays a protective role in preventing liver steatosis and fibrosis, serving as a defender of liver homeostasis in the development of metabolic dysfunction-associated steatotic liver disease (MASLD). However, the regulation of UFMylation in MASLD remains unclear.
View Article and Find Full Text PDFAssessment of Peripheral Neuropathy Using Current Perception Threshold Measurement in Patients with Spinocerebellar Ataxia Type 3.
Cerebellum
January 2025
Department of Neurology, Fujian Key Laboratory of Molecular Neurology, Fujian Institute of Neurology, The First Affiliated Hospital, Fujian Medical University, Key Laboratory of Brain Aging and Neurodegenerative Diseases of Fujian Medical University, 20 Chazhong Road, Fuzhou, 350005, China.
Peripheral neuropathy (PN) identified as a significant contributor to disability in Spinocerebellar ataxia type 3 (SCA3) patients. This study seeks to assess the utility of current perception threshold (CPT) measurements in evaluating PN in individuals with SCA3 and aims to identify factors influencing CPT values in SCA3 and ascertain whether these values correlate with the severity of ataxia. Ninety-four patients diagnosed with SCA3 and 44 healthy controls were recruited for this investigation.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!