G6PD maintains the VSMC synthetic phenotype and accelerates vascular neointimal hyperplasia by inhibiting the VDAC1-Bax-mediated mitochondrial apoptosis pathway
CONCLUSION: Our study showed that the G6PD-VDAC1-Bax axis is a vital switch in VSMC apoptosis and is essential for VSMC phenotypic switching and neointimal hyperplasia, providing mechanistic insight into early VRDs.PMID:38589823 | PMC:PMC11003121 | DOI:10.1186/s11658-024-00566-w (Source: Cellular and Molecular Biology Letters)
Source: Cellular and Molecular Biology Letters - April 8, 2024 Category: Biochemistry Authors: Ting Zhang Rui-Jie Cao Jiang-Ling Niu Zhi-Huan Chen Shi-Qing Mu Tong Cao Jie-Xin Pang Li-Hua Dong Source Type: research

An externally validated clinical-laboratory nomogram for myocardial involvement in adult idiopathic-inflammatory-myopathy patients
ConclusionThis study developed and validated a nomogram model to predict the risk of MI in adult IIMs patients, which can benefit the prediction and early identification of MI as well as timely intervention in these patients. (Source: Clinical Rheumatology)
Source: Clinical Rheumatology - April 8, 2024 Category: Rheumatology Source Type: research

Pathophysiology of Cardiac Amyloidosis
Amyloidosis refers to a heterogeneous group of disorders sharing common pathophysiological mechanisms characterized by the extracellular accumulation of fibrillar deposits consisting of the aggregation of misfolded proteins. Cardiac amyloidosis (CA), usually caused by deposition of misfolded transthyretin or immunoglobulin light chains, is an increasingly recognized cause of heart failure burdened by a poor prognosis. CA manifests with a restrictive cardiomyopathy which progressively leads to biventricular thickening, diastolic and then systolic dysfunction, arrhythmias, and valvular disease. The pathophysiology of CA is m...
Source: Heart Failure Clinics - April 8, 2024 Category: Cardiology Authors: Paolo Morfino, Alberto Aimo, Maria Franzini, Giuseppe Vergaro, Vincenzo Castiglione, Giorgia Panichella, Giuseppe Limongelli, Michele Emdin Source Type: research

Energy metabolism as the hub of advanced non-small cell lung cancer management: a comprehensive view in the framework of predictive, preventive, and personalized medicine
This article provides a detailed overview towards mechanisms and biological pathways involving metabolic reprogramming (MR) with respect to inhibiting the synthesis of biomolecules and blocking common NSCLC metabolic pathways as anti-NSCLC t herapeutic strategies. For instance, mitophagy recycles macromolecules to yield mitochondrial substrates for energy homeostasis and nucleotide synthesis. Histone modification and DNA methylation can predict the onset of diseases, and plasma C7 analysis is an efficient medical service potentially res ulting in an optimized healthcare economy in corresponding areas. The MEMP scoring prov...
Source: EPMA Journal - April 8, 2024 Category: International Medicine & Public Health Source Type: research

Relevance of real-time analyzers to determine mitochondrial quality in endothelial cells and oxidative stress in preeclampsia
Vascul Pharmacol. 2024 Apr 5:107372. doi: 10.1016/j.vph.2024.107372. Online ahead of print.ABSTRACTOxidative stress and mitochondrial dysfunction are important elements for the pathophysiology of preeclampsia (PE), a multisystemic hypertensive syndrome of pregnancy, characterized by endothelial dysfunction and responsible for a large part of maternal and fetal morbidity and mortality worldwide. Researchers have dedicated their efforts to unraveling the intricate ways in which certain molecules influence both energy metabolism and oxidative stress. Exploring established methodologies from existing literature, shows that the...
Source: Vascular Pharmacology - April 7, 2024 Category: Drugs & Pharmacology Authors: Priscila R Nunes Pedro F Oliveira Irene Rebelo Valeria C Sandrim Marco G Alves Source Type: research

Mitochondrial encephalopathies and myopathies: Our tertiary center's experience
We report our center's experience with mitochondrial encephalopathies and myopathies with a cohort of 50 genetically and phenotypically diverse patients followed in the Neurology clinic over the last ten years. Seventeen patients had mitochondrial DNA mutations, presented over a wide range of ages with seizures, feeding difficulties, extraocular movements abnormalities, and had high rates of stroke-like episodes and regression. Twenty-seven patients had nuclear DNA mutations, presented early in life with feeding difficulty, failure-to-thrive, and seizures, and had high proportions of developmental delay, wheelchair depende...
Source: European Journal of Paediatric Neurology - April 7, 2024 Category: Neurology Authors: Can Ozlu Souad Messahel Berge Minassian Saima Kayani Source Type: research

CHCHD10 < sup > S59L/+ < /sup > mouse model: Behavioral and neuropathological features of frontotemporal dementia
Neurobiol Dis. 2024 Apr 5:106498. doi: 10.1016/j.nbd.2024.106498. Online ahead of print.ABSTRACTCHCHD10-related disease causes a spectrum of clinical presentations including mitochondrial myopathy, cardiomyopathy, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). We generated a knock-in mouse model bearing the p.Ser59Leu (S59L) CHCHD10 variant. Chchd10S59L/+ mice have been shown to phenotypically replicate the disorders observed in patients: myopathy with mtDNA instability, cardiomyopathy and typical ALS features (protein aggregation, neuromuscular junction degeneration and spinal motor neuron loss). H...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Emmanuelle C Genin Pauline Pozzo di Borgo Thomas Lorivel Sandrine Hugues M élissa Farinelli Alessandra Mauri-Crouzet Fran çoise Lespinasse Lucas Godin V éronique Paquis-Flucklinger Agn ès Petit-Paitel Source Type: research

Increased molar ratio of free fatty acids to albumin in blood as cause and early biomarker for the development of cataracts and Alzheimer's disease
Exp Eye Res. 2024 Apr 5:109888. doi: 10.1016/j.exer.2024.109888. Online ahead of print.ABSTRACTCataracts and Alzheimer's disease (AD) are closely linked and are associated with aging and with systemic diseases that increase the molar ratio of free fatty acids to albumin (mFAR) in the blood. From the results of our earlier studies on the development of senile cataracts and from results recently published in the literature on the pathogenesis of Alzheimer's disease, we suggest that there is a common lipotoxic cascade for both diseases, explaining the strong connection between aging, an elevated mFAR in the blood, cataract fo...
Source: Experimental Eye Research - April 7, 2024 Category: Opthalmology Authors: Dietmar Glaesser Martin Iwig Source Type: research

Relevance of real-time analyzers to determine mitochondrial quality in endothelial cells and oxidative stress in preeclampsia
Vascul Pharmacol. 2024 Apr 5:107372. doi: 10.1016/j.vph.2024.107372. Online ahead of print.ABSTRACTOxidative stress and mitochondrial dysfunction are important elements for the pathophysiology of preeclampsia (PE), a multisystemic hypertensive syndrome of pregnancy, characterized by endothelial dysfunction and responsible for a large part of maternal and fetal morbidity and mortality worldwide. Researchers have dedicated their efforts to unraveling the intricate ways in which certain molecules influence both energy metabolism and oxidative stress. Exploring established methodologies from existing literature, shows that the...
Source: Vascular Pharmacology - April 7, 2024 Category: Drugs & Pharmacology Authors: Priscila R Nunes Pedro F Oliveira Irene Rebelo Valeria C Sandrim Marco G Alves Source Type: research

Increased molar ratio of free fatty acids to albumin in blood as cause and early biomarker for the development of cataracts and Alzheimer's disease
Exp Eye Res. 2024 Apr 5:109888. doi: 10.1016/j.exer.2024.109888. Online ahead of print.ABSTRACTCataracts and Alzheimer's disease (AD) are closely linked and are associated with aging and with systemic diseases that increase the molar ratio of free fatty acids to albumin (mFAR) in the blood. From the results of our earlier studies on the development of senile cataracts and from results recently published in the literature on the pathogenesis of Alzheimer's disease, we suggest that there is a common lipotoxic cascade for both diseases, explaining the strong connection between aging, an elevated mFAR in the blood, cataract fo...
Source: Experimental Eye Research - April 7, 2024 Category: Opthalmology Authors: Dietmar Glaesser Martin Iwig Source Type: research

CHCHD10 < sup > S59L/+ < /sup > mouse model: Behavioral and neuropathological features of frontotemporal dementia
Neurobiol Dis. 2024 Apr 5:106498. doi: 10.1016/j.nbd.2024.106498. Online ahead of print.ABSTRACTCHCHD10-related disease causes a spectrum of clinical presentations including mitochondrial myopathy, cardiomyopathy, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). We generated a knock-in mouse model bearing the p.Ser59Leu (S59L) CHCHD10 variant. Chchd10S59L/+ mice have been shown to phenotypically replicate the disorders observed in patients: myopathy with mtDNA instability, cardiomyopathy and typical ALS features (protein aggregation, neuromuscular junction degeneration and spinal motor neuron loss). H...
Source: Neurobiology of Disease - April 7, 2024 Category: Neurology Authors: Emmanuelle C Genin Pauline Pozzo di Borgo Thomas Lorivel Sandrine Hugues M élissa Farinelli Alessandra Mauri-Crouzet Fran çoise Lespinasse Lucas Godin V éronique Paquis-Flucklinger Agn ès Petit-Paitel Source Type: research

Relevance of real-time analyzers to determine mitochondrial quality in endothelial cells and oxidative stress in preeclampsia
Vascul Pharmacol. 2024 Apr 6;155:107372. doi: 10.1016/j.vph.2024.107372. Online ahead of print.ABSTRACTOxidative stress and mitochondrial dysfunction are important elements for the pathophysiology of preeclampsia (PE), a multisystemic hypertensive syndrome of pregnancy, characterized by endothelial dysfunction and responsible for a large part of maternal and fetal morbidity and mortality worldwide. Researchers have dedicated their efforts to unraveling the intricate ways in which certain molecules influence both energy metabolism and oxidative stress. Exploring established methodologies from existing literature, shows that...
Source: Vascular Pharmacology - April 7, 2024 Category: Drugs & Pharmacology Authors: Priscila R Nunes Pedro F Oliveira Irene Rebelo Valeria C Sandrim Marco G Alves Source Type: research

Mitochondrial encephalopathies and myopathies: Our tertiary center's experience
We report our center's experience with mitochondrial encephalopathies and myopathies with a cohort of 50 genetically and phenotypically diverse patients followed in the Neurology clinic over the last ten years. Seventeen patients had mitochondrial DNA mutations, presented over a wide range of ages with seizures, feeding difficulties, extraocular movements abnormalities, and had high rates of stroke-like episodes and regression. Twenty-seven patients had nuclear DNA mutations, presented early in life with feeding difficulty, failure-to-thrive, and seizures, and had high proportions of developmental delay, wheelchair depende...
Source: European Journal of Paediatric Neurology - April 7, 2024 Category: Neurology Authors: Can Ozlu Souad Messahel Berge Minassian Saima Kayani Source Type: research

Increased molar ratio of free fatty acids to albumin in blood as cause and early biomarker for the development of cataracts and Alzheimer's disease
Exp Eye Res. 2024 Apr 5:109888. doi: 10.1016/j.exer.2024.109888. Online ahead of print.ABSTRACTCataracts and Alzheimer's disease (AD) are closely linked and are associated with aging and with systemic diseases that increase the molar ratio of free fatty acids to albumin (mFAR) in the blood. From the results of our earlier studies on the development of senile cataracts and from results recently published in the literature on the pathogenesis of Alzheimer's disease, we suggest that there is a common lipotoxic cascade for both diseases, explaining the strong connection between aging, an elevated mFAR in the blood, cataract fo...
Source: Experimental Eye Research - April 7, 2024 Category: Opthalmology Authors: Dietmar Glaesser Martin Iwig Source Type: research

GSE229460 Adaptive epigenetic regulation of neuronal metabolism by a mitochondrial redox signal following complex I inhibition
Contributor : Marius W BaekenSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensDifferent signaling pathways connect the mitochondrion with the transcriptional machinery in the nucleus. Redox events are thought to play a substantial role along this axis, however, many open questions about their specificity, quantitative importance and mode of action remain. Here, we have employed subtoxic doses of the complex I inhibitor MPP+ in human neuronal LUHMES cells to characterize the contribution of scavengeable redox signals to mito-nuclear communication. MPP+ evoked a broadly targeted transcri...
Source: GEO: Gene Expression Omnibus - April 7, 2024 Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research