Potential small effector molecules restoring cellular defects due to sialic acid biosynthetic enzyme deficiency: Pathological relevance to GNE myopathy
Biochem Pharmacol. 2024 Apr 9:116199. doi: 10.1016/j.bcp.2024.116199. Online ahead of print.ABSTRACTGNEM (GNE Myopathy) is a rare neuromuscular disease caused due to biallelic mutations in sialic acid biosynthetic GNE enzyme (UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine Kinase). Recently direct or indirect role of GNE in other cellular functions have been elucidated. Hyposialylation of IGF-1R leads to apoptosis due to mitochondrial dysfunction while hyposialylation of β1 integrin receptor leads to altered F-actin assembly, disrupted cytoskeletal organization and slow cell migration. Other cellular defects in pr...
Source: Biochemical Pharmacology - April 11, 2024 Category: Drugs & Pharmacology Authors: Fluencephila Mashangva Jyoti Oswalia Shagun Singh Ranjana Arya Source Type: research
Mechanical stress induced mitochondrial dysfunction in cardiovascular diseases: Novel mechanisms and therapeutic targets
Biomed Pharmacother. 2024 Apr 10;174:116545. doi: 10.1016/j.biopha.2024.116545. Online ahead of print.ABSTRACTCardiovascular diseases (CVDs) are the leading cause of mortality worldwide. Others and our studies have shown that mechanical stresses (forces) including shear stress and cyclic stretch, occur in various pathological conditions, play significant roles in the development and progression of CVDs. Mitochondria regulate the physiological processes of cardiac and vascular cells mainly through adenosine triphosphate (ATP) production, calcium flux and redox control while promote cell death through electron transport comp...
Source: Atherosclerosis - April 11, 2024 Category: Cardiology Authors: He Ren Weiyi Hu Tao Jiang Qingping Yao Yingxin Qi Kai Huang Source Type: research
Mechanical stress induced mitochondrial dysfunction in cardiovascular diseases: Novel mechanisms and therapeutic targets
Biomed Pharmacother. 2024 Apr 10;174:116545. doi: 10.1016/j.biopha.2024.116545. Online ahead of print.ABSTRACTCardiovascular diseases (CVDs) are the leading cause of mortality worldwide. Others and our studies have shown that mechanical stresses (forces) including shear stress and cyclic stretch, occur in various pathological conditions, play significant roles in the development and progression of CVDs. Mitochondria regulate the physiological processes of cardiac and vascular cells mainly through adenosine triphosphate (ATP) production, calcium flux and redox control while promote cell death through electron transport comp...
Source: Biomedicine and pharmacotherapy = Biomedecine and pharmacotherapie - April 11, 2024 Category: Drugs & Pharmacology Authors: He Ren Weiyi Hu Tao Jiang Qingping Yao Yingxin Qi Kai Huang Source Type: research
Innovative Approaches to Alzheimer's Therapy: Harnessing the Power of Heterocycles, Oxidative Stress Management, and Nanomaterial Drug Delivery System
Ageing Res Rev. 2024 Apr 9:102298. doi: 10.1016/j.arr.2024.102298. Online ahead of print.ABSTRACTAlzheimer's disease (AD) presents a complex pathology involving amyloidogenic proteolysis, neuroinflammation, mitochondrial dysfunction, and cholinergic deficits. Oxidative stress exacerbates AD progression through pathways like macromolecular peroxidation, mitochondrial dysfunction, and metal ion redox potential alteration linked to amyloid-beta (Aβ). Despite limited approved medications, heterocyclic compounds have emerged as promising candidates in AD drug discovery. This review highlights recent advancements in synthetic h...
Source: Ageing Research Reviews - April 11, 2024 Category: Genetics & Stem Cells Authors: Mohammad Umar Yasir Rehman Subiya Ambreen Sayed Md Mumtaz Mohd Shaququzzaman Mohammad Mumtaz Alam Ruhi Ali Source Type: research
The neuroprotective effects of Ferulic acid in toxin-induced models of Parkinson's disease: a review
Ageing Res Rev. 2024 Apr 9:102299. doi: 10.1016/j.arr.2024.102299. Online ahead of print.ABSTRACTParkinson's disease is predominantly caused by dopaminergic neuron loss in the substantia nigra pars compacta and the accumulation of alpha-synuclein protein. Though the general consensus is that several factors, such as aging, environmental factors, mitochondrial dysfunction, accumulations of neurotoxic alpha-synuclein, malfunctions of the lysosomal and proteasomal protein degradation systems, oxidative stress, and neuroinflammation, are involved in the neurodegeneration process of Parkinson's disease, the precise mechanism by...
Source: Ageing Research Reviews - April 11, 2024 Category: Genetics & Stem Cells Authors: Samira Hassani Abolghasem Esmaeili Source Type: research
Exosomal Tenascin-C Primes Macrophage Pyroptosis Amplifying Aberrant Inflammation during Sepsis-Induced Acute Lung Injury
Transl Res. 2024 Apr 9:S1931-5244(24)00078-1. doi: 10.1016/j.trsl.2024.04.001. Online ahead of print.ABSTRACTSepsis-induced acute lung injury (ALI) is a serious complication of sepsis and the predominant cause of death. Exosomes released by lung tissue cells critically influence the progression of ALI during sepsis by modulating the inflammatory microenvironment. However, the molecular mechanisms by which exosome-mediated intercellular signaling exacerbates ALI in septic infection remain undefined. Our study found increased levels of exosomal Tenascin-C (TNC) in the plasma of both patients and mice with ALI, showing a stro...
Source: Translational Research : the journal of laboratory and clinical medicine - April 11, 2024 Category: Laboratory Medicine Authors: Ting Gong Xuedi Zhang Xiaolei Liu Yinfeng Ye Zhiyuan Tian Shuang Yin Min Zhang Jing Tang Youtan Liu Source Type: research
Potential small effector molecules restoring cellular defects due to sialic acid biosynthetic enzyme deficiency: Pathological relevance to GNE myopathy
Biochem Pharmacol. 2024 Apr 9;223:116199. doi: 10.1016/j.bcp.2024.116199. Online ahead of print.ABSTRACTGNEM (GNE Myopathy) is a rare neuromuscular disease caused due to biallelic mutations in sialic acid biosynthetic GNE enzyme (UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine Kinase). Recently direct or indirect role of GNE in other cellular functions have been elucidated. Hyposialylation of IGF-1R leads to apoptosis due to mitochondrial dysfunction while hyposialylation of β1 integrin receptor leads to altered F-actin assembly, disrupted cytoskeletal organization and slow cell migration. Other cellular defects i...
Source: Biochemical Pharmacology - April 11, 2024 Category: Drugs & Pharmacology Authors: Fluencephila Mashangva Jyoti Oswalia Shagun Singh Ranjana Arya Source Type: research
Mechanical stress induced mitochondrial dysfunction in cardiovascular diseases: Novel mechanisms and therapeutic targets
Biomed Pharmacother. 2024 Apr 10;174:116545. doi: 10.1016/j.biopha.2024.116545. Online ahead of print.ABSTRACTCardiovascular diseases (CVDs) are the leading cause of mortality worldwide. Others and our studies have shown that mechanical stresses (forces) including shear stress and cyclic stretch, occur in various pathological conditions, play significant roles in the development and progression of CVDs. Mitochondria regulate the physiological processes of cardiac and vascular cells mainly through adenosine triphosphate (ATP) production, calcium flux and redox control while promote cell death through electron transport comp...
Source: Biomedicine and pharmacotherapy = Biomedecine and pharmacotherapie - April 11, 2024 Category: Drugs & Pharmacology Authors: He Ren Weiyi Hu Tao Jiang Qingping Yao Yingxin Qi Kai Huang Source Type: research
Innovative Approaches to Alzheimer's Therapy: Harnessing the Power of Heterocycles, Oxidative Stress Management, and Nanomaterial Drug Delivery System
Ageing Res Rev. 2024 Apr 9:102298. doi: 10.1016/j.arr.2024.102298. Online ahead of print.ABSTRACTAlzheimer's disease (AD) presents a complex pathology involving amyloidogenic proteolysis, neuroinflammation, mitochondrial dysfunction, and cholinergic deficits. Oxidative stress exacerbates AD progression through pathways like macromolecular peroxidation, mitochondrial dysfunction, and metal ion redox potential alteration linked to amyloid-beta (Aβ). Despite limited approved medications, heterocyclic compounds have emerged as promising candidates in AD drug discovery. This review highlights recent advancements in synthetic h...
Source: Ageing Research Reviews - April 11, 2024 Category: Genetics & Stem Cells Authors: Mohammad Umar Yasir Rehman Subiya Ambreen Sayed Md Mumtaz Mohd Shaququzzaman Mohammad Mumtaz Alam Ruhi Ali Source Type: research
The neuroprotective effects of ferulic acid in toxin-induced models of Parkinson's disease: A review
Ageing Res Rev. 2024 Apr 9;97:102299. doi: 10.1016/j.arr.2024.102299. Online ahead of print.ABSTRACTParkinson's disease is predominantly caused by dopaminergic neuron loss in the substantia nigra pars compacta and the accumulation of alpha-synuclein protein. Though the general consensus is that several factors, such as aging, environmental factors, mitochondrial dysfunction, accumulations of neurotoxic alpha-synuclein, malfunctions of the lysosomal and proteasomal protein degradation systems, oxidative stress, and neuroinflammation, are involved in the neurodegeneration process of Parkinson's disease, the precise mechanism...
Source: Ageing Research Reviews - April 11, 2024 Category: Genetics & Stem Cells Authors: Samira Hassani Abolghasem Esmaeili Source Type: research
C9ORF72 patient-derived endothelial cells drive blood-brain barrier disruption and contribute to neurotoxicity
AbstractThe blood-brain barrier (BBB) serves as a highly intricate and dynamic interface connecting the brain and the bloodstream, playing a vital role in maintaining brain homeostasis. BBB dysfunction has been associated with multiple neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS); however, the role of the BBB in neurodegeneration is understudied. We developed an ALS patient-derived model of the BBB by using cells derived from 5 patient donors carryingC9ORF72 mutations. Brain microvascular endothelial-like cells (BMEC-like cells) derived fromC9ORF72-ALS patients showed altered gene expression, c...
Source: Fluids and Barriers of the CNS - April 11, 2024 Category: Neuroscience Source Type: research
Expedited Exome Re-analysis following Deep Phenotyping and Muscle Biopsy in Suspected Mitochondrial Disorder
Exome sequencing (ES) is a useful tool in diagnosing suspected mitochondrial disease but can miss pathogenic variants for several reasons. Additional testing, such as muscle biopsy or biochemical testing, can be helpful in exome-negative cases. (Source: Pediatric Neurology)
Source: Pediatric Neurology - April 11, 2024 Category: Neurology Authors: Elizabeth Pickup, Steven A. Moore, Pim Suwannarat, Christina Grant, Nicholas Ah Mew, Andrea Gropman, Kuntal Sen Tags: Short Communication Source Type: research
Replication and Transcription of Human Mitochondrial DNA
Annu Rev Biochem. 2024 Apr 9. doi: 10.1146/annurev-biochem-052621-092014. Online ahead of print.ABSTRACTMammalian mitochondrial DNA (mtDNA) is replicated and transcribed by phage-like DNA and RNA polymerases, and our understanding of these processes has progressed substantially over the last several decades. Molecular mechanisms have been elucidated by biochemistry and structural biology and essential in vivo roles established by cell biology and mouse genetics. Single molecules of mtDNA are packaged by mitochondrial transcription factor A into mitochondrial nucleoids, and their level of compaction influences the initiatio...
Source: Annual Review of Biochemistry - April 10, 2024 Category: Biochemistry Authors: Maria Falkenberg Nils-G öran Larsson Claes M Gustafsson Source Type: research
MITOCHONDRIAL IMPORT STRESS and PINK1-MEDIATED MITOPHAGY: THE ROLE of the PINK1-TOMM-TIMM23 SUPERCOMPLEX
Autophagy. 2024 Apr 10. doi: 10.1080/15548627.2024.2340399. Online ahead of print.ABSTRACTMutations in the PINK1 kinase cause Parkinson disease (PD) through physiological processes that are not yet fully elucidated. PINK1 kinase accumulates selectively on damaged mitochondria, where it recruits the E3 ubiquitin ligase PRKN/Parkin to mediate mitophagy. Upon mitochondrial import failure, PINK1 accumulates in association with the translocase of outer mitochondrial membrane (TOMM). However, the molecular basis of this PINK1 accumulation on the TOMM complex remain elusive. We recently demonstrated that TIMM23 (translocase of th...
Source: Autophagy - April 10, 2024 Category: Cytology Authors: Mohamed A Eldeeb Armaan Fallahi Andrea Soumbasis Andrew N Bayne Jean-Fran çois Trempe Edward A Fon Source Type: research
Targeting VDAC: A potential therapeutic approach for mitochondrial dysfunction in Alzheimer's disease
This article aims to provide an overview of the role of VDAC in mitochondrial dysfunction, its association with Alzheimer's disease, and the potential of targeting VDAC for developing novel therapeutic interventions. Understanding the involvement of VDAC in Alzheimer's disease may pave the way for the development of effective treatments that can restore mitochondrial function and halt disease progression.PMID:38599511 | DOI:10.1016/j.brainres.2024.148920 (Source: Brain Research)
Source: Brain Research - April 10, 2024 Category: Neurology Authors: Yaqian Yang Xiaotao Jia Xinmao Yang Jie Wang Yan Fang Xiaoping Ying Meiqian Zhang Jing Wei Yanfang Pan Source Type: research
