Research on minors who have a close family member with amyotrophic lateral sclerosis (ALS) is scarce. This study aims to analyze the relationships between reflective function and wellbeing among such children, considering their reflective function, representations of death, and behavioral problems with the following instruments: Reflective Functioning Questionnaire, Testoni Death Representation Scale for Children, Positive and Negative Affect Schedule for Children, and Strengths and Difficulties Questionnaire. Participants were 248 minors divided into the target group (38 children-16 females, 22 males-7-18 years old (M = 11.61, SD = 2.97)) and the control group (210 students-120 females, 90 males 9-14 years old (M = 11.17, SD = 1.33)). Results showed that the target group exhibited more negative affect and hyperactivity. However, they also showed less uncertainty in their mental states. The opportunity to support these minors is discussed.
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http://dx.doi.org/10.3390/children10010140 | DOI Listing |
CNS Neurosci Ther
January 2025
Jiujiang Clinical Precision Medicine Research Center, Jiujiang, Jiangxi, China.
Background: Adenosine deaminase action on RNA 1 (ADAR1) can convert the adenosine in double-stranded RNA (dsRNA) molecules into inosine in a process known as A-to-I RNA editing. ADAR1 regulates gene expression output by interacting with RNA and other proteins; plays important roles in development, including growth; and is linked to innate immunity, tumors, and central nervous system (CNS) diseases.
Results: In recent years, the role of ADAR1 in tumors has been widely discussed, but its role in CNS diseases has not been reviewed.
Sci Rep
January 2025
Department of Neurology, Feinberg School of Medicine, Northwestern University, 303 E. Chicago Ave, Chicago, IL, 60611, USA.
Corticospinal motor neurons (CSMN), located in the motor cortex of the brain, are one of the key components of the motor neuron circuitry. They are in part responsible for the initiation and modulation of voluntary movement, and their degeneration is the hallmark for numerous diseases, such as amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia, and primary lateral sclerosis. Cortical hyperexcitation followed by in-excitability suggests the early involvement of cortical dysfunction in ALS pathology.
View Article and Find Full Text PDFSci Rep
January 2025
Department of Protein Science, SciLifeLab, KTH Royal Institute of Technology, Stockholm, Sweden.
Accurate diagnosis and monitoring of neurodegenerative diseases require reliable biomarkers. Cerebrospinal fluid (CSF) proteins are promising candidates for reflecting brain pathology; however, their diagnostic utility may be compromised by natural variability between individuals, weakening their association with disease. Here, we measured the levels of 69 pre-selected proteins in cerebrospinal fluid using antibody-based suspension bead array technology in a multi-disease cohort of 499 individuals with neurodegenerative disorders including Alzheimer's disease (AD), behavioral variant frontotemporal dementia, primary progressive aphasias, amyotrophic lateral sclerosis (ALS), corticobasal syndrome, primary supranuclear palsy, along with healthy controls.
View Article and Find Full Text PDFNat Commun
January 2025
Department of Neurology, Columbia University Irving Medical Center, New York, NY, USA.
Mitochondrial function is modulated by its interaction with the endoplasmic reticulum (ER). Recent research indicates that these contacts are disrupted in familial models of amyotrophic lateral sclerosis (ALS). We report here that this impairment in the crosstalk between mitochondria and the ER impedes the use of glucose-derived pyruvate as mitochondrial fuel, causing a shift to fatty acids to sustain energy production.
View Article and Find Full Text PDFBiochim Biophys Acta Mol Cell Res
January 2025
CSIR-Centre for Cellular and Molecular Biology, Hyderabad 500007, India; Florida Research and Innovation Center, Cleveland Clinic, Port Saint Lucie, FL 34987, USA. Electronic address:
Optineurin/OPTN is an adapter protein that plays a crucial role in mediating many cellular functions, including autophagy, vesicle trafficking, and various signalling pathways. Mutations of OPTN are linked with neurodegenerative disorders, glaucoma, and amyotrophic lateral sclerosis (ALS). Recent work has shown that OPTN provides cytoprotection from many types of stress, including oxidative stress, endoplasmic reticulum stress, protein homeostasis stress, tumour necrosis factor α, and microbial infection.
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