Mitochondrial tRNA mutations in 887 Chinese Subjects with Hearing Loss
Publication date: Available online 10 March 2020Source: MitochondrionAuthor(s): Jing Zheng, Xiaohui Bai, Yun Xiao, Yanchun Ji, Feilong Meng, Maerhaba Aishanjiang, Yinglong Gao, Haibo Wang, Yong Fu, Min-Xin Guan (Source: Mitochondrion)
Source: Mitochondrion - March 11, 2020 Category: Biochemistry Source Type: research

Platelet mtDNA content and leukocyte count influence whole blood mtDNA content
Publication date: Available online 7 March 2020Source: MitochondrionAuthor(s): Hanjoo B. Shim, Omair Arshad, Izabella Gadawska, Hélène C.F. Côté, Anthony Y.Y. Hsieh (Source: Mitochondrion)
Source: Mitochondrion - March 7, 2020 Category: Biochemistry Source Type: research

Causes and consequences of mitochondrial proteome size variation in animals
Publication date: May 2020Source: Mitochondrion, Volume 52Author(s): Viraj Muthye, Dennis V. Lavrov (Source: Mitochondrion)
Source: Mitochondrion - March 7, 2020 Category: Biochemistry Source Type: research

Combination therapy with astaxanthin and epidermal neural crest stem cells improves motor impairments and activates mitochondrial biogenesis in a rat model of spinal cord injury
Publication date: Available online 6 March 2020Source: MitochondrionAuthor(s): Leila Mohaghegh Shalmani, Neda Valian, Safura Pournajaf, Fatemeh Abbaszadeh, Leila Dargahi, Masoumeh Jorjani (Source: Mitochondrion)
Source: Mitochondrion - March 7, 2020 Category: Biochemistry Source Type: research

Alpha lipoic acid and Vitamin E improve atorvastatin-induced mitochondrial dysfunctions in rats
Publication date: Available online 28 February 2020Source: MitochondrionAuthor(s): Hatice Eser Faki, Bunyamin Tras, Kamil Uney (Source: Mitochondrion)
Source: Mitochondrion - February 29, 2020 Category: Biochemistry Source Type: research

The ERMES (Endoplasmic Reticulum and Mitochondria Encounter Structures) mediated functions in fungi
Publication date: Available online 24 February 2020Source: MitochondrionAuthor(s): Deepika Kundu, Ritu Pasrija (Source: Mitochondrion)
Source: Mitochondrion - February 25, 2020 Category: Biochemistry Source Type: research

Causes and consequences of mitochondrial proteome size-variation in animals
Publication date: Available online 25 February 2020Source: MitochondrionAuthor(s): Viraj Muthye, Dennis V. Lavrov (Source: Mitochondrion)
Source: Mitochondrion - February 25, 2020 Category: Biochemistry Source Type: research

Development of a nanoparticle that releases nucleic acids in response to a mitochondrial environment
Publication date: Available online 22 February 2020Source: MitochondrionAuthor(s): Yuma Yamada, Yutaka Fukuda, Daisuke Sasaki, Minako Maruyama, Hideyoshi HarashimaAbstractThe delivery of nucleic acids targeting mutant mtDNA represent a potential strategy for addressing a variety of mitochondria-related diseases. We previously developed a MITO-Porter, a nano carrier that is capable of delivering nanoparticles of nucleic acids to mitochondria of human cells. Here, we report on an investigation of a series of nanoparticles formed with various poly cationic peptides that can release nucleic acids in response to a mitochondrial...
Source: Mitochondrion - February 24, 2020 Category: Biochemistry Source Type: research

Expanding the phenotype of mitochondrial disease: novel pathogenic variant in ISCA1 leading to instability of the iron-sulfur cluster in the protein
We report a patient carrying a novel pathogenic variant p.(Tyr101Cys) in ISCA1 leading to MMDS type 5. He initially presented a psychomotor regression with loss of gait and language skills and a tetrapyramidal spastic syndrome. Biochemical analysis of patient fibroblasts revealed impaired lipoic acid synthesis and decreased activities of complex I and II of respiratory chain. While ISCA1 is involved in mitochondrial machinery of iron-sulfur cluster biogenesis, these dysfunctions are secondary to impaired maturation of mitochondrial proteins containing the [4Fe-4S] clusters. Expression and purification of the human ISCA1 sh...
Source: Mitochondrion - February 23, 2020 Category: Biochemistry Source Type: research

Mitochondrial Dysfunction: An Emerging Link in the Pathophysiology of Polycystic Ovary Syndrome
Publication date: Available online 17 February 2020Source: MitochondrionAuthor(s): Pallavi Shukla, Srabani MukherjeeAbstractPolycystic ovary syndrome (PCOS) is a common endocrine disorder characterized by irregular menstrual cycles, hyperandrogenism and subfertility. Due to its complex manifestation, the pathogenic mechanism of PCOS is not well defined. Cumulative effect of altered genetic and epigenetic factors along with environmental factors may play a role in the manifestation of PCOS leading to systemic malfunction. With failure of genome-wide association study (GWAS) and other studies performed on nuclear genome to p...
Source: Mitochondrion - February 18, 2020 Category: Biochemistry Source Type: research

Characterization of background noise in MiSeq MPS data when sequencing human mitochondrial DNA from various sample sources and library preparation methods
Publication date: Available online 14 February 2020Source: MitochondrionAuthor(s): Jennifer A. McElhoe, Mitchell M. HollandAbstractImproved resolution of massively parallel sequencing (MPS) allows for the characterization of mitochondrial (mt) DNA heteroplasmy to levels previously unattainable with traditional sequencing approaches. An essential criterion for the reporting of heteroplasmy is the ability of the MPS method to distinguish minor sequence variants (MSVs) from system noise, or error. Therefore, an assessment of the background noise in the MPS method is desirable to identify the point at which reliable data can b...
Source: Mitochondrion - February 15, 2020 Category: Biochemistry Source Type: research

Prophylaxis of Mitochondrial Dysfunction Caused by Cellular Decompression from Hyperbaric Exposure
Publication date: Available online 8 February 2020Source: MitochondrionAuthor(s): Abhay Ranganathan, Shawn Owiredu, David H. Jang, David M. EckmannAbstractMitochondrial dysfunction occurring in response to cellular perturbations can include altered mitochondrial motility and bioenergetic function having intracellular heterogeneity. Exogenous mitochondrial directed therapy may correct these dysfunctions. Using in vitro approaches, we find that cell perturbations induced by rapid decompression from hyperbaric conditions with specific gas exposures has differential effects on mitochondrial motility, inner membrane potential, ...
Source: Mitochondrion - February 9, 2020 Category: Biochemistry Source Type: research

qMGR: a new approach for quantifying mitochondrial genome rearrangement
We presented qMGR, a new approach for large-scale quantifying mitogenome rearrangements considering a single gene as a structural unit. Compared to a reference arrangement, qMGR accumulates the changes of two nearest neighbor genes to calculate rearrangement score (RS) and rearrangement frequency (RF) of each single gene in the mitogenomes of a given taxonomic group. By accumulating RS of all genes in one genome, qMGR was developed to calculate each mitogenome rearrangement score, which can be used as a quantitative feature of the mitogenome rearrangement. Based on the frequency of rearrangement of each gene, qMGR can furt...
Source: Mitochondrion - February 9, 2020 Category: Biochemistry Source Type: research

Coxfa4l3, a novel mitochondrial electron transport chain Complex 4 subunit protein, switches from Coxfa4 during spermatogenesis
Publication date: Available online 8 February 2020Source: MitochondrionAuthor(s): Masahiro Endou, Kaito Yoshida, Makoto Hirota, Chika Nakajima, Atsumi Sakaguchi, Naoto Komatsubara, Yasuyuki KuriharaAbstractWe identified Coxfa4l3, previously called C15orf48 or Nmes1, as a novel accessory protein of Complex IV of the mitochondrial electron transport chain (ETC). Amino acid sequence comparison, the intracellular localization and the protein expression data showed that the protein is the third isoform of Coxfa4 and the expression of Coxfa4 and Coxfa4l3 proteins during spermatogenesis showed a mutually exclusive pattern, implyi...
Source: Mitochondrion - February 9, 2020 Category: Biochemistry Source Type: research

Mitochondrial-associated impairments of Temozolomide on Neural Stem/Progenitor Cells and Hippocampal Neurons
In this study, we examined the mitochondrial-mediated mechanisms involving TMZ-induced neural damage in primary rat neural stem/progenitor cells (NSC) and hippocampal neurons. TMZ inhibited mtDNA replication and transcription of mitochondrial genes (ND1 and Cyt b) in NSC by 24 h, whereas the effect of TMZ on neuronal mtDNA transcription was less pronounced. Transmission electron microscopy imaging revealed mitochondrial degradation in TMZ-treated NSC. Acute TMZ exposure (4 h) caused a rapid reduction in dendritic branching and loss of postsynaptic density-95 (PSD95) puncta on dendrites. Longer TMZ exposure impaired mitocho...
Source: Mitochondrion - February 9, 2020 Category: Biochemistry Source Type: research