Pan-neuronal expression of human mutant SOD1 in Drosophila impairs survival and motor performance, induces early neuroinflammation and chromosome aberrations
Biochim Biophys Acta Mol Basis Dis. 2024 Apr 22:167192. doi: 10.1016/j.bbadis.2024.167192. Online ahead of print.ABSTRACTSeveral mutations in the SOD1 gene encoding for the antioxidant enzyme Superoxide Dismutase 1, are associated with amyotrophic lateral sclerosis, a rare and devastating disease characterized by motor neuron degeneration and patients' death within 2-5 years from diagnosis. Motor neuron loss and related symptomatology manifest mostly in adult life and, to date, there is still a gap of knowledge on the precise cellular and molecular events preceding neurodegeneration. To deepen our awareness of the early ph...
Source: Biochimica et Biophysica Acta - April 24, 2024 Category: Biochemistry Authors: Francesco Liguori Francesca Alberti Susanna Amadio Daniela Francesca Angelini Eleonora Pilesi Giuseppe Vitale Giulia Tesoriere Giovanna Borsellino Fiammetta Vern ì Cinzia Volont é Source Type: research
Identifying the Unmet Needs of People Living With Amyotrophic Lateral Sclerosis: A National Survey to Inform Interdisciplinary Palliative Care
Discussion: Interdisciplinary palliative care teams may enhance ALS care by promoting advance care planning behaviors (particularly discussing one's wishes with healthcare providers), providing interventions to improve quality of life, and supporting pALS in navigating challenges related to physical intimacy.PMID:38657132 | DOI:10.1177/10499091241248653 (Source: The American Journal of Hospice and Palliative Care)
Source: The American Journal of Hospice and Palliative Care - April 24, 2024 Category: Palliative Care Authors: Karla T Washington Charlton A Mechling Kyle A Pitzer Samuel Maiser Ambereen K Mehta Source Type: research
Real-time ultrasound-guided sacral plexus block combined with mild sedation for hemorrhoidectomy and hemorrhoidal artery ligation in a patient with amyotrophic lateral sclerosis: a case report
ConclusionsReal-time ultrasound-guided sacral plexus block combined with mild sedation may be an effective and safe technique in patients with amyotrophic lateral sclerosis undergoing hemorrhoidectomy and hemorrhoidal artery ligation. (Source: Journal of Medical Case Reports)
Source: Journal of Medical Case Reports - April 24, 2024 Category: General Medicine Source Type: research
C/EBP β: A transcription factor associated with the irreversible progression of Alzheimer's disease
CONCLUSION: The overexpression of C/EBPβ accelerates the irreversible progression of AD pathophysiology. Additionally, C/EBPβ plays a crucial role in mediating multiple pathways linked to AD pathology, some of which engender vicious cycles, leading to the establishment of feedback mechanisms. To sum up, targeting C/EBPβ could hold promise as a therapeutic strategy not only for AD but also for other degenerative diseases.PMID:38644578 | PMC:PMC11033503 | DOI:10.1111/cns.14721 (Source: CNS Neuroscience and Therapeutics)
Source: CNS Neuroscience and Therapeutics - April 22, 2024 Category: Neuroscience Authors: Qing Yao Chubing Long Pengcheng Yi Guangyong Zhang Wei Wan Xiuqin Rao Jun Ying Weidong Liang Fuzhou Hua Source Type: research
C/EBP β: A transcription factor associated with the irreversible progression of Alzheimer's disease
CONCLUSION: The overexpression of C/EBPβ accelerates the irreversible progression of AD pathophysiology. Additionally, C/EBPβ plays a crucial role in mediating multiple pathways linked to AD pathology, some of which engender vicious cycles, leading to the establishment of feedback mechanisms. To sum up, targeting C/EBPβ could hold promise as a therapeutic strategy not only for AD but also for other degenerative diseases.PMID:38644578 | PMC:PMC11033503 | DOI:10.1111/cns.14721 (Source: CNS Neuroscience and Therapeutics)
Source: CNS Neuroscience and Therapeutics - April 22, 2024 Category: Neuroscience Authors: Qing Yao Chubing Long Pengcheng Yi Guangyong Zhang Wei Wan Xiuqin Rao Jun Ying Weidong Liang Fuzhou Hua Source Type: research
Amyotrophic lateral sclerosis stratification: unveiling patterns with virome, inflammation, and metabolism molecules
AbstractAmyotrophic lateral sclerosis (ALS) is an untreatable and clinically heterogeneous condition primarily affecting motor neurons. The ongoing quest for reliable biomarkers that mirror the disease status and progression has led to investigations that extend beyond motor neurons ’ pathology, encompassing broader systemic factors such as metabolism, immunity, and the microbiome. Our study contributes to this effort by examining the potential role of microbiome-related components, including viral elements, such as torque tenovirus (TTV), and various inflammatory factors, in ALS. In our analysis of serum samples from 10...
Source: Journal of Neurology - April 21, 2024 Category: Neurology Source Type: research
Advance Care Planning in a Multidisciplinary Amyotrophic Lateral Sclerosis Clinic
1. Discuss the aspects of advance care planning that are unique or complex in patients with ALS.2. Discuss the effectiveness of integrated palliative care and social work to increase rates of ACP completion in a multidisciplinary, urban ALS clinic. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - April 19, 2024 Category: Palliative Care Authors: Astrid Grouls, James Orengo, Minh B. Nguyen Source Type: research
GSE264018 Polystyrene nanoparticles trigger aberrant condensation of TDP-43 and amyotrophic lateral sclerosis-like symptoms
Contributors : Hang Sun ; Bingwei Yang ; Qiong Li ; Yang SongSeries Type : Expression profiling by high throughput sequencingOrganism : Mus musculusAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the dysfunction and progressive death of cerebral and spinal motor neurons. Preliminary epidemiological research has hinted at a relationship between environmental risks and the escalation of ALS, but the underlying reasons remain mostly mysterious. Here, we show that nano-size polystyrene plastics (PS) induce ALS-like symptoms and illustrate the related molecular mechanism. When exposed to ...
Source: GEO: Gene Expression Omnibus - April 19, 2024 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Mus musculus Source Type: research
Abundant transcriptomic alterations in the human cerebellum of patients with a C9orf72 repeat expansion
AbstractThe most prominent genetic cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) is a repeat expansion in the geneC9orf72. Importantly, the transcriptomic consequences of theC9orf72 repeat expansion remain largely unclear. Here, we used short-read RNA sequencing (RNAseq) to profile the cerebellar transcriptome, detecting alterations in patients with aC9orf72 repeat expansion. We focused on the cerebellum, since keyC9orf72-related pathologies are abundant in this neuroanatomical region, yet TDP-43 pathology and neuronal loss are minimal. Consistent with previous work, we show...
Source: Acta Neuropathologica - April 19, 2024 Category: Neurology Source Type: research
Choroid plexus enlargement in amyotrophic lateral sclerosis patients and its correlation with clinical disability and blood-CSF barrier permeability
ConclusionOur study first demonstrates CP enlargement in vivo in ALS patients, and continues to suggest an important pathogenetic role for CP abnormalities in ALS. Moreover, assessing CP volume is likely a noninvasive and easy-to-implement approach for screening BCSFB dysfunction in ALS patients. (Source: Fluids and Barriers of the CNS)
Source: Fluids and Barriers of the CNS - April 17, 2024 Category: Neuroscience Source Type: research
Choroid plexus enlargement in amyotrophic lateral sclerosis patients and its correlation with clinical disability and blood-CSF barrier permeability
Using in vivo neuroimaging techniques, growing evidence has demonstrated that the choroid plexus (CP) volume is enlarged in patients with several neurodegenerative diseases, including Alzheimer ’s disease and P... (Source: Cerebrospinal Fluid Research)
Source: Cerebrospinal Fluid Research - April 17, 2024 Category: Neurology Authors: Tingjun Dai, Jianwei Lou, Deyuan Kong, Jinyu Li, Qingguo Ren, Yujing Chen, Sujuan Sun, Yan Yun, Xiaohan Sun, Yiru Yang, Kai Shao, Wei Li, Yuying Zhao, Xiangshui Meng, Chuanzhu Yan, Pengfei Lin & hellip; Tags: Research Source Type: research
Correction
Volume 25, Issue 3-4, May 2024, Page (II)-(II) . (Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration)
Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - April 17, 2024 Category: Neurology Source Type: research
Correction to: Cognitive function in amyotrophic lateral sclerosis: a cross ‑sectional and prospective pragmatic clinical study with review of the literature
(Source: Neurological Sciences)
Source: Neurological Sciences - April 16, 2024 Category: Neurology Source Type: research
Using an expanded algorithm to estimate prevalence of amyotrophic lateral sclerosis in U.S. and UK
ConclusionsThe expanded algorithm enabled the identification of a large population with ALS, or possible ALS, and the estimation of ALS prevalence in MarketScan and CPRD. (Source: Neurological Sciences)
Source: Neurological Sciences - April 16, 2024 Category: Neurology Source Type: research
Longitudinal Changes in the Retinal Nerve Fiber Layer Thickness in Amyotrophic Lateral Sclerosis and Parkinson's Disease: A Comparative Study
CONCLUSIONS: The RNFL thickness is a promising biomarker of disease progression in patients with ALS but not in those with PD, which has a slower disease progression.PMID:38627230 | DOI:10.3988/jcn.2023.0353 (Source: Journal of Clinical Neurology)
Source: Journal of Clinical Neurology - April 16, 2024 Category: Neurology Authors: Sharifah Azira Taufik Norlina Ramli Ai Huey Tan Shen-Yang Lim Mohd Taufiq Abdul Ghani Nortina Shahrizaila Source Type: research
