Editorial Board
Publication date: March 2020Source: Mitochondrion, Volume 51Author(s): (Source: Mitochondrion)
Source: Mitochondrion - February 8, 2020 Category: Biochemistry Source Type: research
Calcium Overload Decreases Net Free Radical Emission in Cardiac Mitochondria
In this study, we quantitatively demonstrate that, on the contrary, calcium does not stimulate free radical production but suppresses it. Isolated mitochondria from guinea pig hearts were energized with a variety of substrates and exposed to calcium concentrations designed to induce moderate calcium overload conditions associated with ischemia/reperfusion injury but do not elicit the well-known mitochondrial permeability transition phenomenon. Metabolic function and free radical emission were simultaneously quantified using high-resolution respirometry and fluorimetry. In parallel, membrane potential, high amplitude swelli...
Source: Mitochondrion - January 24, 2020 Category: Biochemistry Source Type: research
Detection of mitochondrial DNA variants at low level heteroplasmy in pediatric CNS and extra-CNS solid tumors with three different enrichment methods
This study demonstrates that LRPCR and SureSelect enrichment, but not REPLI-g, followed by NGS are accurate methods for studying the mtDNA variations at low heteroplasmy, which may be applied to studying mtDNA mutations in cancer. (Source: Mitochondrion)
Source: Mitochondrion - January 21, 2020 Category: Biochemistry Source Type: research
MMPdb and MitoPredictor: tools for facilitating comparative analysis of animal mitochondrial proteomes
In this study, we developed two resources to address these challenges: 1] the Metazoan Mitochondrial Proteome Database (MMPdb), which consolidates data on experimentally-characterized mitochondrial proteomes of vertebrate and invertebrate model organisms, and 2] MitoPredictor, a novel machine-learning tool for prediction of mitochondrial proteins in animals. MMPdb allows comparative analysis of animal mitochondrial proteomes by integrating results from orthology analysis, prediction of mitochondrial targeting signals, protein domain analysis, and Gene Ontology analysis. Additionally, for mammalian mitochondrial proteins, M...
Source: Mitochondrion - January 21, 2020 Category: Biochemistry Source Type: research
Redox Homeostasis, Oxidative Stress and Mitophagy
Publication date: Available online 20 January 2020Source: MitochondrionAuthor(s): Carla Garza-Lombó, Aglaia Pappa, Mihalis I. Panayiotidis, Rodrigo FrancoAbstractAutophagy is a ubiquitous homeostatic mechanism for the degradation or turnover of cellular components. Degradation of mitochondria via autophagy (mitophagy) is involved in a number of physiological processes including cellular homeostasis, differentiation and aging. Upon stress or injury, mitophagy prevents the accumulation of damaged mitochondria and the increased steady state levels of reactive oxygen species leading to oxidative stress and cell death. A numbe...
Source: Mitochondrion - January 21, 2020 Category: Biochemistry Source Type: research
Cell stress management by the mitochondrial LonP1 protease – Insights into mitigating developmental, oncogenic and cardiac stress
Publication date: March 2020Source: Mitochondrion, Volume 51Author(s): Sundararajan Venkatesh, Carolyn K. SuzukiAbstractMitochondrial LonP1 is an essential stress response protease that mediates mitochondrial proteostasis, metabolism and bioenergetics. Homozygous and compound heterozygous variants in the LONP1 gene encoding the LonP1 protease have recently been shown to cause a diverse spectrum of human pathologies, ranging from classical mitochondrial disease phenotypes, profound neurologic impairment and multi-organ dysfunctions, some of which are uncommon to mitochondrial disorders. In this review, we focus primarily on...
Source: Mitochondrion - January 14, 2020 Category: Biochemistry Source Type: research
Relative Telomere Length and Mitochondrial DNA Copy Number Variation with Age: Association with Plasma Folate and Vitamin B12
In conclusion, folate and vitamin B12 may delay aging by preventing the reduction in rTL length and mtCN. (Source: Mitochondrion)
Source: Mitochondrion - January 12, 2020 Category: Biochemistry Source Type: research
Revealing mitogenome-wide DNA methylation and RNA editing of three Ascomycotina fungi using SMRT sequencing
Publication date: Available online 7 January 2020Source: MitochondrionAuthor(s): Chaoxia Wang, Jianhua Feng, Yujiao Chen, Dongmei Li, Li Liu, Yuqian Wu, Shujun Zhang, Simiao Du, Yaozhou ZhangAbstractBeauveria bassiana, Cordyceps militaris and Ophiocordyceps sinensis (Ascomycotina) are traditional Chinese medicines. Here, mitogenomes of these three Ascomycotina fungi were sequenced and de-novo assembled using single-molecule real-time sequencing. The results showed that their complete mitogenomes were 31,258, 31,854 and 157,584 bp, respectively, with sequencing depth approximately 278,760×, 326,283× and 69,385×. Types of...
Source: Mitochondrion - January 8, 2020 Category: Biochemistry Source Type: research
Editorial: Special Issue on Mitochondrial Fidelity
Publication date: Available online 7 January 2020Source: MitochondrionAuthor(s): Oleh Khalimonchuk (Source: Mitochondrion)
Source: Mitochondrion - January 7, 2020 Category: Biochemistry Source Type: research
LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity
We report a patient heterozygous for a de novo mutation in LONP1: c.901C>T,p.R301W presenting as a neonate with seizures, encephalopathy, pachygyria and microcephaly. Assays of respiratory chain activity in muscle showed complex II-III function at 8% of control. Functional studies in patient fibroblasts showed a signature of dysfunction that included significant decreases in known proteolytic targets of LONP1 (TFAM, PINK1, phospho-PDH E1α) as well as loss of mitochondrial ribosome subunits MRPL44 and MRPL11 with concomitant decreased activity and level of protein subunits of oxidative phosphorylation complexes I and IV. T...
Source: Mitochondrion - January 7, 2020 Category: Biochemistry Source Type: research
Mitochondrial Haplogroups and Lifespan in a Population Isolate
The objective of the current study is to replicate the prior findings in an independent cohort that includes both sexes and younger ages. By determining and cross-referencing the mtDNA genotypes of a culturally homogeneous population isolate to the lifespans of their ancestors, we found that between 1930 and 1939, haplogroup H compared to haplogroup U carriers had a modestly lengthened lifespan (3 years) past 60 years (hazard ratio 2.35; CI95 1.41-3.90; p-value: 0.0029). The lifespan-lengthening association was apparent in both sexes but only after the age of 60. Our results provide further support for the role of mitochon...
Source: Mitochondrion - December 28, 2019 Category: Biochemistry Source Type: research
Positive regulation of human PINK1 and Parkin gene expression by nuclear respiratory factor 1
In conclusion, NRF-1 has a positive regulatory effect on the transcription of PINK1 and Parkin genes, and involves in mitochondrial quality control through regulating PINK1/Parkin-mediated mitophagy. (Source: Mitochondrion)
Source: Mitochondrion - December 27, 2019 Category: Biochemistry Source Type: research
Cerebrospinal-fluid ATP is inappropriate as a biomarker of disease severity and treatment response in MELAS
Publication date: Available online 24 December 2019Source: MitochondrionAuthor(s): Josef FinstererAbstractNone, letter (Source: Mitochondrion)
Source: Mitochondrion - December 26, 2019 Category: Biochemistry Source Type: research
Perspective: Cell Danger Response Biology—The New Science that Connects Environmental Health with Mitochondria and the Rising Tide of Chronic Illness
Publication date: Available online 23 December 2019Source: MitochondrionAuthor(s): Robert K. NaviauxAbstractThis paper is written for non-specialists in mitochondrial biology to provide access to an important area of science that has broad implications for all people. The cell danger response (CDR) is a universal response to environmental threat or injury. Once triggered, healing cannot be completed until the choreographed stages of the CDR are returned to an updated state of readiness. Although the CDR is a cellular response, it has the power to change human thought and behavior, child development, physical fitness and re...
Source: Mitochondrion - December 24, 2019 Category: Biochemistry Source Type: research
Editorial Board
Publication date: January 2020Source: Mitochondrion, Volume 50Author(s): (Source: Mitochondrion)
Source: Mitochondrion - December 24, 2019 Category: Biochemistry Source Type: research
