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mitochondrial biogenesis

Jonathan G Van Vranken, Sara M Nowinski, Katie J Clowers, Mi-Young Jeong, Yeyun Ouyang, Jordan A Berg, Jeremy P Gygi, Steven P Gygi, Dennis R Winge, Jared Rutter
The electron transport chain (ETC) is an important participant in cellular energy conversion, but its biogenesis presents the cell with numerous challenges. To address these complexities, the cell utilizes ETC assembly factors, which include the LYR protein family. Each member of this family interacts with the mitochondrial acyl carrier protein (ACP), the scaffold protein upon which the mitochondrial fatty acid synthesis (mtFAS) pathway builds fatty acyl chains from acetyl-CoA. We demonstrate that the acylated form of ACP is an acetyl-CoA-dependent allosteric activator of the LYR protein family used to stimulate ETC biogenesis...
August 16, 2018: Molecular Cell
Jin Yu, Hong Zhu, Saeid Taheri, Stephen Perry, Mark S Kindy
The consumption of fruits and vegetables appears to help with maintaining an adequate level of exercise and improves endurance. However, the mechanisms that are involved in this process are not well understood. In the current study, the impact of diets enriched in fruits and vegetables (GrandFusion® ) on exercise endurance was examined in a mouse model. GrandFusion (GF) diets increased mitochondrial DNA and enzyme activity, while they also stimulated mitochondrial mRNA synthesis in vivo. GF diets increased both the mRNA expression of factors involved in mitochondrial biogenesis, peroxisome proliferator-activated receptor gamma coactivator 1 alpha (PGC-1α), mitochondrial transcription factor A (Tfam), estrogen-related receptor alpha (ERRα), nuclear respiratory factor 1 (NRF-1), cytochrome c oxidase IV (COXIV) and ATP synthase (ATPsyn)...
August 16, 2018: Nutrients
Sylvia J Horne, Jessica Vasquez, Yiqing Guo, Victoria Ly, Sian Piret, Alexandria Leonardo, Jason Ling, Monica P Revelo, Daniel Bogenhagen, Vincent W Yang, John C He, Sandeep K Mallipattu
Mitochondrial injury is uniformly observed in several murine models as well as in individuals with diabetic kidney disease (DKD). Although emerging evidence has highlighted the role of key transcriptional regulators in mitochondrial biogenesis, little is known about the regulation of mitochondrial cytochrome c oxidase (COX) assembly in the podocyte under diabetic conditions. We recently reported a critical role of the zinc finger, Krüppel-like factor 6 (KLF6), in maintaining mitochondrial function and preventing apoptosis in a proteinuric murine model...
August 16, 2018: Diabetes
J-G Cai, L-M Luo, H Tang, L Zhou
One of the end-products of ROS-induced peroxidation, malondialdehyde (MDA), induces the cross-links in proteins, which leads to perturbation of the physiological functions of cells and contributes to abnormal biological regulation and various disorders. Taurine (2-aminoethanesulfonic acid, Tau) aids in adjusting normal physiological functions to confer stress resistance. The protective effects of Tau against MDA stress in vitro or in vivo were reported previously. In this study, we had investigated the protective effects of taurine on viability, oxidative stress levels and mitochondrial biogenesis in mouse muscle C2C12 cells undergoing MDA induced stress...
July 2018: Molekuliarnaia Biologiia
Yong Chen, Haining Gao, Wenling Ye
Heavy-ion irradiation-induced nuclear DNA damage and mutations have been studied comprehensively. However, there is no information about the deleterious effect of heavy-ion irradiation on mitochondrial DNA (mtDNA). In this study, 2 typical mtDNA mutations were examined, including 4977 deletions and D310 point mutations. The 4977 deletions were quantified by real-time polymerase chain reaction, and D310 point mutations were analyzed by direct sequencing and a specific enzyme digestion genotyping method. Results showed that carbon ions radiation can induce temporal fluctuation of mtDNA 4977 deletions in 72 hours after irradiation, while survived clones were free from this deletion...
July 2018: Dose-response: a Publication of International Hormesis Society
Zhenji Gan, Tingting Fu, Daniel P Kelly, Rick B Vega
Skeletal muscle fitness and plasticity is an important determinant of human health and disease. Mitochondria are essential for maintaining skeletal muscle energy homeostasis by adaptive re-programming to meet the demands imposed by a myriad of physiologic or pathophysiological stresses. Skeletal muscle mitochondrial dysfunction has been implicated in the pathogenesis of many diseases, including muscular dystrophy, atrophy, type 2 diabetes, and aging-related sarcopenia. Notably, exercise counteracts the effects of many chronic diseases on skeletal muscle mitochondrial function...
August 14, 2018: Cell Research
Anju Shukla, Parneet Kaur, Katta M Girisha
Iron-sulfur cluster assembly 1 (ISCA1) is one of the essential proteins operating in the mitochondrial iron-sulfur (Fe-S) cluster biogenesis pathway. We reported the variant c.259G > A [p.(Glu87Lys)] in homozygous state in exon 4 of the ISCA1 gene as the likely cause of multiple mitochondrial dysfunction syndrome 5 in a previous publication. We now report the third patient with the same phenotype and variant, further supporting the possibility of a founder event. Our observation confirms the clinical presentation associated with a probable founder variant in this condition...
September 2018: Journal of Pediatric Genetics
Paola Pontieri, Hans Hartings, Marco Di Salvo, Domenica R Massardo, Mario De Stefano, Graziano Pizzolante, Roberta Romano, Jacopo Troisi, Angelica Del Giudice, Pietro Alifano, Luigi Del Giudice
A considerable body of evidence links together mitochondrial dysfunctions, toxic action of metalloid oxyanions, and system and neurodegenerative disorders. In this study we have used the model yeast Saccharomyces cerevisiae to investigate the genetic determinants associated with tellurite resistance/sensitivity. Nitrosoguanidine-induced K2 TeO3 -resistant mutants were isolated, and one of these mutants, named Sc57-Te5 R , was characterized. Both random spore analysis and tetrad analysis and growth of heterozygous (TeS /Te5 R ) diploid from Sc57-Te5 R mutant revealed that nuclear and recessive mutation(s) was responsible for the resistance...
August 13, 2018: Scientific Reports
Junnan Ma, Seok Yong Kang, Xianglong Meng, An Na Kang, Jong Hun Park, Yong-Ki Park, Hyo Won Jung
With the aging process, a loss of skeletal muscle mass and dysfunction related to metabolic syndrome is observed in older people. Yams are commonly use in functional foods and medications with various effects. The present study was conducted to investigate the effects of rhizome extract of Dioscorea batatas (Dioscoreae Rhizoma, Chinese yam) and its bioactive compound, allantoin, on myoblast differentiation and mitochondrial biogenesis in skeletal muscle cells. Yams were extracted in water and allantoin was analyzed by high performance liquid chromatography (HPLC)...
August 13, 2018: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
Heather L Petrick, Kaitlyn M J H Dennis, Paula M Miotto
No abstract text is available yet for this article.
August 13, 2018: Journal of Physiology
Hojun Lee, Kijeong Kim, Boa Kim, Junchul Shin, Sudarsan Rajan, Jingwei Wu, Xiongwen Chen, Michael D Brown, Sukho Lee, Joon-Young Park
KEY POINTS: Referring to the muscle memory theory, previously trained muscles acquire strength and volume much faster than naive muscles. Using extreme experimental models such as synergist ablation or steroid administration, previous studies have demonstrated that the number of nuclei increases when a muscle becomes enlarged, which serves as a cellular muscle memory mechanism for the muscle. In the present study, we found that, when rats were subjected to physiologically relevant resistance training, the number of myonuclei increased and was retained during a long-term detraining period...
August 12, 2018: Journal of Physiology
Takeshi Yoneshiro, Mami Matsushita, Masayuki Saito
Since the rediscovery of brown adipose tissue (BAT) in humans, its energy-dissipating ability has been well-recognized. The negative correlations of BAT activity with adiposity and insulin sensitivity provided an obvious rationale for discerning reliable and practical strategies for stimulating BAT. Though cold exposure or use of pharmacological adrenomimetics can activate BAT, they may have adverse effects. Therefore, determining alternative stimulants of BAT with lower risks such as commonly used food ingredients is highly desirable...
August 12, 2018: Handbook of Experimental Pharmacology
Huan Liu, Siying Li, Xiaohui Liu, Yuling Chen, Haiteng Deng
SIRT3 is a NAD+-dependent mitochondrial protein deacetylase implicated in the regulation of central metabolism and mitochondrial proteostasis. SIRT3 is downregulated in clear cell renal cell carcinoma (ccRCC), which is the most common form of renal cancer. Although ccRCC is characterized by a typical Warburg-like phenotype, mitochondrial dysfunction and elevated fat deposition, it is unknown whether SIRT3 plays a role in tumorigenesis and the development of this disease. In the present study, we found that SIRT3 overexpression and knockdown had opposing effects on the growth of ccRCC cells, decreasing and increasing the rate of cell proliferation, respectively...
August 10, 2018: Journal of Proteome Research
Zheying Min, Qian Gao, Xiumei Zhen, Yong Fan, Tao Tan, Rong Li, Yue Zhao, Yang Yu
BACKGROUND: Polycystic ovary syndrome (PCOS) is a common endocrine and metabolic disorder that affects female fertility. However, with the lack of a corresponding research model, the pathology mechanism of PCOS is poorly understood. Induced pluripotent stem cell (iPSC) technology has been recognized as means to generate patient-specific stem cells for disease modeling. METHODS: The mRNA abundance of iPSCs was analyzed by RNA microarray and real-time polymerase chain reaction (RT-PCR)...
August 9, 2018: Stem Cell Research & Therapy
Braulio Vargas Möller-Hergt, Andreas Carlström, Katharina Stephan, Axel Imhof, Martin Ott
Mitochondrial gene expression in Saccharomyces cerevisiae is responsible for the production of highly hydrophobic subunits of the oxidative phosphorylation system. Membrane insertion occurs co-translationally on membrane bound mitochondrial ribosomes. Here, by employing a systematic mass-spectrometric-based approach, we discovered the previously uncharacterized membrane protein Mrx15 that interacts via a soluble C-terminal domain with the large ribosomal subunit. Mrx15 contacts mitochondrial translation products during their synthesis and plays, together with the ribosome receptor Mba1, an overlapping role in co-translational protein insertion...
August 9, 2018: Molecular Biology of the Cell
Adi Drori, Anna Permyakova, Rivka Hadar, Shiran Udi, Alina Nemirovski, Joseph Tam
AIMS: To evaluate the specific role of the endocannabinoid/CB1 R system in modulating mitochondrial dynamics in the metabolically active renal proximal tubular cells (RPTCs). MATERIALS AND METHODS: We utilized mitochondrialy-targeted GFP in live cells (wild-type and null for the CB1 R), and electron microscopy in kidney sections of RPTC-CB1 R-/- mice and their littermate controls. In both in vitro and in vivo conditions, we assessed the ability of CB1 R agonism or fatty acid flux to modulate mitochondrial architecture and function...
August 9, 2018: Diabetes, Obesity & Metabolism
Peng Liao, Meifang Liao, Ling Li, Bie Tan, Yulong Yin
This study was conducted to determine the effect of 200 ng mL-1 and 2000 ng mL-1 deoxynivalenol (DON) on apoptosis, barrier function, nutrient transporter gene expression, and free amino acid variation as well as on mitochondrial biogenesis and function-related gene expression in the intestinal porcine epithelial cell line J2 (IPEC-J2) for 6 h, 12 h, and 24 h. Exposure to 200 ng mL-1 DON inhibited the cell viability and promoted cell cycle progression from the G2/M phase to the S phase ( P < 0.05). The data showed that the IPEC-J2 cell content of free amino acids, such as valine, methionine, leucine, and phenylalanine, was increased ( P < 0...
November 1, 2017: Toxicology Research
Jason A Chen
BACKGROUND: Recent advances in genetics have provided insights into important inherited causes of Parkinson's disease (PD), but the underlying biological mechanisms are still incompletely understood. Gene expression studies have pointed toward the dysregulation of neuroinflammation, mitochondrial function, and protein degradation pathways. OBJECTIVE: We aimed to identify groups of dysregulated genes in PD. METHODS: In order to increase statistical power and control for potential confounders, we re-analyzed transcriptomic data from PD patients and model systems, integrating additional genomic data using a systems biology approach...
August 8, 2018: Neuro-degenerative Diseases
Seungyoon B Yu, Gulcin Pekkurnaz
To maintain homeostasis, every cell must constantly monitor its energy level and appropriately adjust energy, in the form of ATP, production rates based on metabolic demand. Continuous fulfillment of this energy demand depends on the ability of cells to sense, metabolize, and convert nutrients into chemical energy. Mitochondria are the main energy conversion sites for many cell types. Cellular metabolic states dictate the mitochondrial size, shape, function and positioning. Mitochondrial shape varies from singular discrete organelles to interconnected reticular networks within cells...
August 5, 2018: Journal of Molecular Biology
Kai Kaarniranta, Jakub Kajdanek, Jan Morawiec, Elzbieta Pawlowska, Janusz Blasiak
PGC-1α (peroxisome proliferator-activated receptor gamma coactivator 1-alpha) is a transcriptional coactivator of many genes involved in energy management and mitochondrial biogenesis. PGC-1α expression is associated with cellular senescence, organismal aging, and many age-related diseases, including AMD (age-related macular degeneration), an important global issue concerning vision loss. We and others have developed a model of AMD pathogenesis, in which stress-induced senescence of retinal pigment epithelium (RPE) cells leads to AMD-related pathological changes...
August 7, 2018: International Journal of Molecular Sciences
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