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Condition: Mitochondrial Disease

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Total 454 results found since Jan 2013.

C-Raf Colocalizes with DAPK in the Mitochondria
This study identified the interaction of C-Raf with S308 phosphorylated DAPK (pDAPKS308), which together became colocalized in the mitochondria to facilitate mitochondrial remodeling. Combined use of the Raf inhibitors sorafenib and GW5074 had synergistic anticancer effects in vitro and in vivo, but targeted mitochondrial function, rather than the canonical Raf signaling pathway. C-Raf depletion in knockout MEFC-Raf−/− or siRNA knockdown ACHN renal cancer cells abrogated the cytotoxicity of combination therapy. Crystal structure simulation showed that GW5074 bound to C-Raf and induced a C-Raf conformational change that...
Source: Cancer Research - August 31, 2015 Category: Cancer & Oncology Authors: Tsai, Y.-T., Chuang, M.-J., Tang, S.-H., Wu, S.-T., Chen, Y.-C., Sun, G.-H., Hsiao, P.-W., Huang, S.-M., Lee, H.-J., Yu, C.-P., Ho, J.-Y., Lin, H.-K., Chen, M.-R., Lin, C.-C., Chang, S.-Y., Lin, V. C., Yu, D.-S., Cha, T.-L. Tags: Therapeutics, Targets, and Chemical Biology Source Type: research

Elevation of Sestrin-2 expression attenuates Sevoflurane induced neurotoxicity
In this study, our results indicated that administration of Sevoflurane elevated the gene and protein expression of Sestrin-2 in a dose dependent manner in human neuroblastoma M17 cells. It was shown that silence of Sestrin-2 by small RNA interference (siRNA) ominously exacerbated the increase in intracellular ROS and reduction of SOD activity induced by Sevoflurane treatment. Notably, knockdown of Sestrin-2 in M17 cells significantly increases the number of apoptotic cells after treatment with Sevoflurane. Mechanistically, we also found that Sevoflurane treatment resulted in a reduced amount of the cytosolic anti-apoptoti...
Source: Metabolic Brain Disease - September 5, 2015 Category: Neurology Source Type: research

Mitochondrial DNA has a pro-inflammatory role in AMD
This study contributes to our understanding of the potential pro-inflammatory role of mtDNA in the pathogenesis of AMD. Graphical abstract
Source: Biochimica et Biophysica Acta (BBA) Molecular Cell Research - September 7, 2015 Category: Molecular Biology Source Type: research

UDCA exerts beneficial effect on mitochondrial dysfunction in LRRK2G2019S carriers and in vivo
Conclusion: There is clear preclinical impairment of mitochondrial function in NM-LRRK2G2019S that is distinct from the mitochondrial impairment observed in parkin-related PD. The beneficial effect of UDCA on mitochondrial function in both NM-LRRK2G2019S and M-LRRK2G2019S as well as on the function of dopaminergic neurons expressing LRRK2G2019S suggests that UDCA is a promising drug for future neuroprotective trials.
Source: Neurology - September 7, 2015 Category: Neurology Authors: Mortiboys, H., Furmston, R., Bronstad, G., Aasly, J., Elliott, C., Bandmann, O. Tags: All Movement Disorders, Parkinson's disease/Parkinsonism ARTICLE Source Type: research

The mitochondrial voltage-dependent anion channel 1 in tumor cells
This article is part of a Special Issue entitled: Membrane channels and transporters in cancers. Graphical abstract
Source: Biochimica et Biophysica Acta (BBA) Biomembranes - September 10, 2015 Category: Biochemistry Source Type: research

NICOTINAMIDE N-METHYLTRANSFERASE increases complex I activity IN SH-SY5Y cells via SIRTUIN-3.
Abstract Nicotinamide N-methyltransferase (NNMT, E.C. 2.1.1.1) N-methylates nicotinamide to 1-methylnicotinamide. We have previously shown that NNMT is significantly overexpressed in the brains of patients who have died of Parkinson's disease, and others have shown that NNMT is significantly overexpressed in a variety of diseases ranging from cancer to hepatic cirrhosis. In vitro overexpression has revealed many cytoprotective effects of NNMT, in particular increased complex I activity and ATP synthesis. Although this appears to be mediated by an increase in 1-methylnicotinamide production, the molecular mechanism...
Source: Biochemical and Biophysical Research communications - October 8, 2015 Category: Biochemistry Authors: Liu KY, Mistry RJ, Aguirre CA, Fasouli ES, Thomas MG, Klamt F, Ramsden DB, Parsons RB Tags: Biochem Biophys Res Commun Source Type: research

Abstract B19: MYC induces PLD6 to suppress YAP/TAZ-dependent self-renewal of mammary stem cells
For the maintenance of a given tissue it is absolutely critical to balance proliferation and differentiation of stem cells, progenitor cells and terminally differentiated cells. Perturbations of these finely tuned processes can lead to various diseases such as cancer.One factor that has been implicated in the transition from a stem cell to a progenitor/ transit-amplifying cell is the oncogenic transcription factor MYC. Paradoxically, despite its strong pro-tumorigenic capabilities it has been shown to promote differentiation in several tissues such as the skin or the hematopoietic system.To identify critical pathways that ...
Source: Molecular Cancer Research - October 18, 2015 Category: Cancer & Oncology Authors: Eyss, B. v., Jaenicke, L. A., Wiese, K., Rosenwald, A., Eilers, M. Tags: Myc Beyond Cancer: Poster Presentations - Proffered Abstracts Source Type: research

Cystine accumulation attenuates insulin release from the pancreatic beta-cell due to elevated oxidative stress and decreased ATP levels.
This article is protected by copyright. All rights reserved. PMID: 26482480 [PubMed - as supplied by publisher]
Source: The Journal of Physiology - October 20, 2015 Category: Physiology Authors: McEvoy B, Sumayao R, Slattery C, McMorrow T, Newsholme P Tags: J Physiol Source Type: research

GAPDH Aggregates Promote A{beta} Amyloidogenesis Molecular Bases of Disease
Alzheimer disease (AD) is a progressive neurodegenerative disorder characterized by loss of neurons and formation of pathological extracellular deposits induced by amyloid-β peptide (Aβ). Numerous studies have established Aβ amyloidogenesis as a hallmark of AD pathogenesis, particularly with respect to mitochondrial dysfunction. We have previously shown that glycolytic glyceraldehyde-3-phosphate dehydrogenase (GAPDH) forms amyloid-like aggregates upon exposure to oxidative stress and that these aggregates contribute to neuronal cell death. Here, we report that GAPDH aggregates accelerate Aβ amyloidogenesis and subseque...
Source: Journal of Biological Chemistry - October 23, 2015 Category: Chemistry Authors: Itakura, M., Nakajima, H., Kubo, T., Semi, Y., Kume, S., Higashida, S., Kaneshige, A., Kuwamura, M., Harada, N., Kita, A., Azuma, Y.-T., Yamaji, R., Inui, T., Takeuchi, T. Tags: Neurobiology Source Type: research

BNIP3 promotes calcium and calpain-dependent cell death
Publication date: 1 December 2015 Source:Life Sciences, Volume 142 Author(s): Regina M. Graham, John W. Thompson, Keith A. Webster Aims Loss of cardiac muscle by programmed cell death contributes to the progression of ischemic heart disease. Hypoxia, metabolite waste buildup and energy depletion are components of ischemia which may initiate caspase dependent and independent cell death pathways. Previous work from our laboratory has shown that combined hypoxia with acidosis, a hallmark of ischemia promotes cardiac myocyte injury with increasing severity as the pH declines. Hypoxia-acidosis was demonstrated to activate ...
Source: Life Sciences - October 23, 2015 Category: Biology Source Type: research

Impaired ALDH2 activity decreases the mitochondrial respiration in H9C2 cardiomyocytes.
Abstract Reactive oxygen species (ROS)-mediated reactive aldehydes induce cellular stress. In cardiovascular diseases such as ischemia-reperfusion injury, lipid-peroxidation derived reactive aldehydes such as 4-hydroxy-2-nonenal (4HNE) are known to contribute to the pathogenesis. 4HNE is involved in ROS formation, abnormal calcium handling and more importantly defective mitochondrial respiration. Aldehyde dehydrogenase (ALDH) superfamily contains NAD(P)(+)-dependent isozymes which can detoxify endogenous and exogenous aldehydes into non-toxic carboxylic acids. Therefore we hypothesize that 4HNE afflicts mitochondr...
Source: Cellular Signalling - November 11, 2015 Category: Cytology Authors: Mali VR, Deshpande M, Pan G, Thandavarayan RA, Palaniyandi SS Tags: Cell Signal Source Type: research

Rg1 prevents myocardial hypoxia/reoxygenation injury by regulating mitochondrial dynamics imbalance via modulation of glutamate dehydrogenase and mitofusin 2
Conclusion Rg1 through modulation of GDH and MFN2 maintained mitochondrial dynamics that resulted in protection against H/R-induced cardiomyocyte injury. All these results put forward a new protective mechanism of Rg1 on the therapeutic potential in cardiac I/R disorders.
Source: Mitochondrion - November 24, 2015 Category: Biochemistry Source Type: research

Regulation of mitochondrial oxidative stress by {beta}-arrestins in cultured human cardiac fibroblasts RESEARCH ARTICLE
ABSTRACT Oxidative stress in cardiac fibroblasts (CFs) promotes transformation to myofibroblasts and collagen synthesis leading to myocardial fibrosis, a precursor to heart failure (HF). NADPH oxidase 4 (Nox4) is a major source of cardiac reactive oxygen species (ROS); however, mechanisms of Nox4 regulation are unclear. β-arrestins are scaffold proteins that signal in G-protein-dependent and -independent pathways; for example, in ERK activation. We hypothesize that β-arrestins regulate oxidative stress in a Nox4-dependent manner and increase fibrosis in HF. CFs were isolated from normal and failing adult human le...
Source: DMM Disease Models and Mechanisms - December 4, 2015 Category: Biomedical Science Authors: Philip, J. L., Razzaque, M. A., Han, M., Li, J., Theccanat, T., Xu, X., Akhter, S. A. Tags: RESEARCH ARTICLE Source Type: research

VDAC1 Mediates A{beta} Cytotoxicity Cell Biology
In this study we demonstrate the involvement of VDAC1 and a VDAC1 N-terminal peptide (VDAC1-N-Ter) in Aβ cell penetration and cell death induction. Aβ directly interacted with VDAC1 and VDAC1-N-Ter, as monitored by VDAC1 channel conductance, surface plasmon resonance, and microscale thermophoresis. Preincubated Aβ interacted with bilayer-reconstituted VDAC1 and increased its conductance ∼2-fold. Incubation of cells with Aβ resulted in mitochondria-mediated apoptotic cell death. However, the presence of non-cell-penetrating VDAC1-N-Ter peptide prevented Aβ cellular entry and Aβ-induced mitochondria-mediated apoptosi...
Source: Journal of Biological Chemistry - December 25, 2015 Category: Chemistry Authors: Smilansky, A., Dangoor, L., Nakdimon, I., Ben-Hail, D., Mizrachi, D., Shoshan-Barmatz, V. Tags: Molecular Bases of Disease Source Type: research

Abstract A12: Establishment of myeloid lineage cell line that resembles myeloid-derived suppressive cells
Myeloid-derived suppressive cells (MDSCs) are inflammatory cells that play critical roles in promoting cancer growth and metastasis. In order to facilitate characterization of biochemical and cellular mechanisms of MDSCs, it is urgent to establish an "MDSC-like" cell line for pharmacological and immunotherapeutic applications. Lysosomal acid lipase (LAL) is a critical lipid enzyme in the metabolic signaling pathway that hydrolyzes cholesteryl esters (CE) and triglycerides (TG) in lysosomes. In mice, lack of LAL in genetically ablated knockout mice (lal-/-) shows systemic expansion of MDSCs, which directly stimulate cancer ...
Source: Molecular Cancer Research - January 15, 2016 Category: Cancer & Oncology Authors: Yan, C., Ding, X., Wu, L., Du, H. Tags: Cancer Metabolic Pathways: Poster Presentations - Proffered Abstracts Source Type: research