Huntington's disease (HD) is a rare hereditary neurodegenerative disorder characterized by multiple metabolic dysfunctions including defects in mitochondrial homeostasis and functions. Although we have recently reported age-related changes in the respiratory capacities in different brain areas in HD mice, the precise mechanisms of how mitochondria become compromised in HD are still poorly understood. In this study, we investigated mRNA and protein levels of selected subunits of electron transport system (ETS) complexes and ATP-synthase in the cortex and striatum of symptomatic R6/2 mice. Our findings reveal a brain-region-specific differential expression of both nuclear and mitochondrial-encoded ETS components, indicating defects of transcription, translation and/or mitochondrial import of mitochondrial ETS components in R6/2 mouse brains.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8533690 | PMC |
| http://dx.doi.org/10.3390/brainsci11101267 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Electron transfer in polysaccharide monooxygenase catalysis.
Proc Natl Acad Sci U S A
January 2025
California Institute for Quantitative Biosciences, University of California, Berkeley, CA 94720.
Polysaccharide monooxygenase (PMO) catalysis involves the chemically difficult hydroxylation of unactivated C-H bonds in carbohydrates. The reaction requires reducing equivalents and will utilize either oxygen or hydrogen peroxide as a cosubstrate. Two key mechanistic questions are addressed here: 1) How does the enzyme regulate the timely and tightly controlled electron delivery to the mononuclear copper active site, especially when bound substrate occludes the active site? and 2) How does this electron delivery differ when utilizing oxygen or hydrogen peroxide as a cosubstrate? Using a computational approach, potential paths of electron transfer (ET) to the active site copper ion were identified in a representative AA9 family PMO from (PMO9E).
View Article and Find Full Text PDFA multidimensional pan-cancer analysis of NDUFA4L2 and verification of the oncogenic value in colon cancer.
FASEB J
January 2025
Department of General Surgery, Sir Run Run Hospital of Nanjing Medical University, Nanjing, Jiangsu, P. R. China.
NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 (NDUFA4L2) protein is located in the mitochondria and can regulate cell proliferation. Some studies have shown that the high NDUFA4L2 expression is linked with poor prognosis and cancer progression in various patients with cancers. However, the correlation between NDUFA4L2 and pan-cancer is unknown.
View Article and Find Full Text PDFLong-term mitochondrial and metabolic impairment in lymphocytes of subjects who recovered after severe COVID-19.
Cell Biol Toxicol
January 2025
Lab. Genetics and Molecular Bases of Complex Diseases, Health Research Institute of Hospital Clínico San Carlos (IdISSC), 28040, Madrid, Spain.
The underlying mechanisms explaining the differential course of SARS-CoV-2 infection and the potential clinical consequences after COVID-19 resolution have not been fully elucidated. As a dysregulated mitochondrial activity could impair the immune response, we explored long-lasting changes in mitochondrial functionality, circulating cytokine levels, and metabolomic profiles of infected individuals after symptoms resolution, to evaluate whether a complete recovery could be achieved. Results of this pilot study evidenced that different parameters of aerobic respiration in lymphocytes of individuals recuperated from a severe course lagged behind those shown upon mild COVID-19 recovery, in basal conditions and after simulated reinfection, and they also showed altered glycolytic capacity.
View Article and Find Full Text PDFEnhanced Light Transmittance of Electron Transport Layer through Bilayer SnO2 for High-Performance Semitransparent Perovskite Solar Cells.
ChemSusChem
January 2025
North China Electric Power University, Changping district, NO.2, Beinong Road, CHINA.
Semitransparent perovskite solar cells (ST-PSCs) for building-integrated photovoltaics (BIPV) face the challenge of achieving high efficiency due to significant light loss. The SnO2 electron transport layer (ETL), utilized in n-i-p PSCs and prepared via the sol-gel method, is susceptible to aggregation on substrate, resulting in light scattering that diminishes absorption of the perovskite layer. In this study, we propose a strategy that combines atomic layer deposition (ALD) and sol-gel solution to deposit a bilayer SnO2 structure to address these issues.
View Article and Find Full Text PDFNonpolar P-type Conjugated Small Molecules Enable High-Performance Organic Photodetectors for Potential Application in Optical Wireless Communication.
Nano Lett
January 2025
School of Advanced Materials, Shenzhen Graduate School, Peking University, Shenzhen 518055, P. R. China.
The high responsivity and broad spectral sensitivity of organic photodetectors (OPDs) present a bright future of commercialization. However, the relatively high dark current density still limits its development. Herein, two novel nonpolar p-type conjugated small molecules, NSN and NSSN, are synthesized as interface layers to enhance the performance of the OPDs, which not only can tune energy alignments and increase the reverse charge injection barrier but also can reduce the interfacial trap density.
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!