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Condition: Mitochondrial Disease

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Total 454 results found since Jan 2013.

Beneficial effects of PGC-1 α in the substantia nigra of a mouse model of MPTP-induced dopaminergic neurotoxicity.
CONCLUSIONS: Our results indicated that PGC-1α rescues the effects of MPTP-induced mitochondrial dysfunction in C57BL mice. PMID: 31634150 [PubMed - as supplied by publisher]
Source: Aging - October 20, 2019 Category: Biomedical Science Authors: Wang Y, Chen C, Huang W, Huang M, Wang J, Chen X, Ye Q Tags: Aging (Albany NY) Source Type: research

TBX3 deficiency accelerates apoptosis in cardiomyoblasts through regulation of P21 expression
In conclusion, we demonstrated that TBX3 deficiency accelerated apoptosis via directly regulating P21 expression in senescent cardiomyoblasts.
Source: Life Sciences - November 6, 2019 Category: Biology Source Type: research

Inhibition of DNM1L and mitochondrial fission attenuates inflammatory response in fibroblast-like synoviocytes of rheumatoid arthritis.
Abstract Mitochondrial fission and fusion are important for mitochondrial function, and dynamin 1-like protein (DNM1L) is a key regulator of mitochondrial fission. We investigated the effect of mitochondrial fission on mitochondrial function and inflammation in fibroblast-like synoviocytes (FLSs) during rheumatoid arthritis (RA). DNM1L expression was determined in synovial tissues (STs) from RA and non-RA patients. FLSs were isolated from STs and treated with a DNM1L inhibitor (mdivi-1, mitochondrial division inhibitor 1) or transfected with DNM1L-specific siRNA. Mitochondrial morphology, DNM1L expression, cell vi...
Source: J Cell Mol Med - November 20, 2019 Category: Molecular Biology Authors: Wang X, Chen Z, Fan X, Li W, Qu J, Dong C, Wang Z, Ji Z, Li Y Tags: J Cell Mol Med Source Type: research

l-ornithine activates Ca2+ signaling to exert its protective function on human proximal tubular cells.
Abstract Oxidative stress and reactive oxygen species (ROS) generation can be influenced by G-protein coupled receptor (GPCR)-mediated regulation of intracellular Ca2+ ([Ca2+]i) signaling. ROS production are much higher in proximal tubular (PT) cells; in addition, the lack of antioxidants enhances the vulnerability to oxidative damage. Despite such predispositions, PT cells show resiliency, and therefore must possess some inherent mechanism to protect from oxidative damage. While the mechanism in unknown, we tested the effect of l-ornithine, since it is abundantly present in PT luminal fluid and can activate calci...
Source: Cellular Signalling - November 22, 2019 Category: Cytology Authors: Shin S, Gombedza FC, Bandyopadhyay BC Tags: Cell Signal Source Type: research

Mitochondrial carnitine palmitoyltransferase 2 is involved in Nε-(carboxymethyl)-lysine-mediated diabetic nephropathy
Publication date: Available online 12 December 2019Source: Pharmacological ResearchAuthor(s): Jangho Lee, Ju-Yong Hyon, Jin Young Min, Yang Hoon Huh, Hyo Jung Kim, Hayoung Lee, Sung Ho Yun, Chi-Won Choi, Su Jeong Ha, Joon Park, Young-Ho Chung, Hye Gwang Jeong, Sang Keun Ha, Sung Keun Jung, YoonSook Kim, Eun Hee HanAbstractDiabetic nephropathy (DN) is the most common cause of end-stage renal disease in the world. Advanced glycation end products (AGEs) are thought to be involved in the pathogenesis of DN via multifactorial mechanisms including the generation of oxidative stress and overproduction of various growth factors an...
Source: Pharmacological Research - December 13, 2019 Category: Drugs & Pharmacology Source Type: research

Extracellular mitochondrial DNA promote NLRP3 inflammasome activation and induce acute lung injury through TLR9 and NF- κB.
Conclusions: Extracellular mtDNA promote NLRP3 inflammasome activation, acute pulmonary inflammation and injury through TLR9, p38 MAPK and NF-κB pathways. PMID: 31903272 [PubMed]
Source: Journal of Thoracic Disease - January 8, 2020 Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research

Thioredoxin-Interacting Protein (TXNIP) Regulates Parkin/PINK1-mediated Mitophagy in Dopaminergic Neurons Under High-glucose Conditions: Implications for Molecular Links Between Parkinson ’s Disease and Diabetes
In this study, we investigated the roles of thioredoxin-interacting protein (TXNIP) in Parkin/PINK1-mediated mitophagy in dopaminergic (DA) cells under high-glucose (HG) conditions. In streptozotocin-induced diab etic mice, TXNIP was upregulated and autophagy was inhibited in the midbrain, while the loss of DA neurons was accelerated by hyperglycemia. In cultured PC12 cells under HG, TXNIP expression was upregulated and the intracellular reactive oxygen species (ROS) levels increased, leading to cell death. Autophagic flux was further blocked and PINK1 expression was decreased under HG conditions. Parkin expression in the ...
Source: Neuroscience Bulletin - January 13, 2020 Category: Neuroscience Source Type: research

Alterations in α-synuclein and PINK1 expression reduce neurite length and induce mitochondrial fission and Golgi fragmentation in midbrain neurons
This study provides novel evidence that α-synuclein overexpression and PINK1 deletion converge to induce significant increases in Golgi fragmentation and mitochondrial fission in midbrain neurons, that are correlated with decreases in neurite length. This highlights the need for further studies on these converging mechanisms in dopaminergic neurodegeneration in PD.
Source: Neuroscience Letters - January 23, 2020 Category: Neuroscience Source Type: research

Embelin averts MPTP-induced dysfunction in mitochondrial bioenergetics and biogenesis via activation of SIRT1
Publication date: Available online 24 January 2020Source: Biochimica et Biophysica Acta (BBA) - BioenergeticsAuthor(s): Swetha Pavani Rao, Neelam Sharma, Shasi V. KalivendiAbstractParkinson's disease (PD) is a chronic neurodegenerative disease characterized by the death of dopamine neurons of Substantia nigra pars compacta (SNpc) leading to motor deficits. Amongst the mechanisms proposed, mitochondrial dysfunction, reduced complex-I and PGC1α levels were found to correlate with the pathology of PD. As embelin is a natural product with structural resemblance to ubiquinone, exhibits mitochondrial uncoupling and antioxidant ...
Source: Biochimica et Biophysica Acta (BBA) Bioenergetics - January 26, 2020 Category: Biochemistry Source Type: research

Elevated Aldolase 1A, retrogene 1 expression induces cardiac apoptosis in rat experimental autoimmune myocarditis model.
Abstract Acute myocarditis is an unpredictable heart disease, which is caused by inflammation-associated cell death. Although viral infection and drug exposure are known to induce acute myocarditis, the molecular basis for its development remains undefined. Using proteomics and molecular analyses in myosin-induced rat experimental autoimmune myocarditis (EAM), we identified that elevated expression of aldolase 1A, retrogene 1 (Aldoart1) is critical to induce mitochondrial dysfunction and acute myocarditis development. Here, we demonstrate that cardiac cell death is associated with increased expressions of pro-apop...
Source: Canadian Journal of Physiology and Pharmacology - January 29, 2020 Category: Drugs & Pharmacology Authors: Choi S, Chung JH, Nam MH, Bang E, Hong KS, Kim YH, Seo JB, Chi SG Tags: Can J Physiol Pharmacol Source Type: research

Resveratrol protects against nonalcoholic fatty liver disease by improving lipid metabolism and redox homeostasis via the PPAR α pathway
This study investigated the preventive and therapeutic effects of RSV on high-fat diet (HFD)-induced NAFLD in rats and palmitate acid (PA)-induced hepatocyte steatosis in HepG2 cells. Hepatocytes were incubated with inhibitors of peroxisome proliferator-activated receptor α (PPARα) or short interfering RNAs (siRNAs) targeting PPARα, AMP-activated protein kinase (AMPK), and protein kinase A (PKA) to determine the underlying mechanisms. We found that RSV noticeably ameliorated HFD-induced hepatic steatosis in rats and inhibited PA-induced lipid accumulation in HepG2 cells. Moreover, RSV improved lipid metabolism, enhanced...
Source: Applied Physiology, Nutrition, and Metabolism - June 6, 2019 Category: Physiology Authors: Yujie Huang Hedong Lang Ka Chen Yong Zhang Yanxiang Gao Li Ran Long Yi Mantian Mi Qianyong Zhang Source Type: research

Melatonin attenuates doxorubicin-induced cardiotoxicity through preservation of YAP expression.
In this study, we examined the possible protective effects of melatonin on doxorubicin-induced cardiotoxicity and explored the underlying mechanisms related to this process. We found that in vitro doxorubicin treatment significantly decreased H9c2 cell viability and induced apoptosis as manifested by increased TUNEL-positive cells, down-regulation of anti-apoptotic protein Bcl-2, as well as up-regulation of pro-apoptotic protein Bax. This was associated with increased reactive oxygen species (ROS) levels and decreased mitochondrial membrane potentials (MMP). In vivo, five weeks of doxorubicin treatment significantly decrea...
Source: J Cell Mol Med - February 17, 2020 Category: Molecular Biology Authors: Li HR, Wang C, Sun P, Liu DD, Du GQ, Tian JW Tags: J Cell Mol Med Source Type: research

Interaction of α-synuclein and Parkin in iron toxicity on SH-SY5Y cells: implications in the pathogenesis of Parkinson's disease.
This study has demonstrated that iron in varying concentrations (up to 400 µM) causes an increase in α-synuclein content in SH-SY5Y cells associated with mitochondrial depolarization, decreased cellular ATP content and loss of cell viability during incubation up to 96 h. Knocking-down α-synuclein expression prevents cytotoxic actions of iron, which can also be prevented by cyclosporine A (a blocker of mitochondrial permeability transition pore). These results indicate that iron cytotoxicity is mediated by α-synuclein acting on mitochondria. Likewise siRNA mediated knock-down of Parkin causes an  accumulation of  α-s...
Source: The Biochemical Journal - February 27, 2020 Category: Biochemistry Authors: Ganguly U, Banerjee A, Chakrabarti SS, Kaur U, Sen O, Cappai R, Chakrabarti S Tags: Biochem J Source Type: research

The mitochondrial protein PGAM5 suppresses energy consumption in brown adipocytes by repressing expression of uncoupling protein 1 Metabolism
Accumulating evidence suggests that brown adipose tissue (BAT) is a potential therapeutic target for managing obesity and related diseases. PGAM family member 5, mitochondrial serine/threonine protein phosphatase (PGAM5), is a protein phosphatase that resides in the mitochondria and regulates many biological processes, including cell death, mitophagy, and immune responses. Because BAT is a mitochondria-rich tissue, we have hypothesized that PGAM5 has a physiological function in BAT. We previously reported that PGAM5-knockout (KO) mice are resistant to severe metabolic stress. Importantly, lipid accumulation is suppressed i...
Source: Journal of Biological Chemistry - April 23, 2020 Category: Chemistry Authors: Sho Sugawara, Yusuke Kanamaru, Shiori Sekine, Lila Maekawa, Akinori Takahashi, Tadashi Yamamoto, Kengo Watanabe, Takao Fujisawa, Kazuki Hattori, Hidenori Ichijo Tags: Cell Biology Source Type: research

Tetrahydroxy stilbene glycoside alleviated inflammatory damage by mitophagy via AMPK related PINK1/Parkin signaling pathway.
In conclusion, these phenomena suggested that TSG prevented LPS/ATP and Aβ-induced inflammation via AMPK/PINK1/Parkin-dependent enhancement of mitophagy. We found the neuroprotective effect of TSG, suggesting it may be beneficial for AD prevention and treatment by suppressing the activation of inflammation. PMID: 32353422 [PubMed - as supplied by publisher]
Source: Biochemical Pharmacology - April 26, 2020 Category: Drugs & Pharmacology Authors: Gao Y, Li J, Li J, Hu C, Zhang L, Yan J, Li L, Zhang L Tags: Biochem Pharmacol Source Type: research