Read by QxMD icon Read


Sophia Miliotis, Bryan Nicolalde, Mayra Ortega, Jackie Yepez, Andrés Caicedo
Mitochondria play an important role as an intracellular energy plant and signaling organelle. However, mitochondria also exist outside cells where they could mediate cell-to-cell communication, repair and serve as an activator of the immune response. Their effects depend on the mitochondrial state or the form in which it is present, either as a whole functional structure as fragments or only as mitochondrial DNA. Herein, we provide evidence of why extracellular mitochondria and their varying forms are considered regenerative factors or pro-inflammatory activators...
February 13, 2019: Mitochondrion
Olivia V Poole, Chris M Everett, Sonia Gandhi, Silvia Marino, Enrico Bugiardini, Cathy Woodward, Amanda Lam, Ros Quinlivan, Michael G Hanna, Robert D S Pitceathly
Adult-onset Leigh syndrome is a rare but important manifestation of mitochondrial disease. We report a 17 year old female who presented with subacute encephalopathy, brainstem and extrapyramidal signs, raised CSF lactate, and symmetrical hyperintensities in the basal ganglia on T2-weighted cerebral MRI. The presence of cytochrome c oxidase deficient fibres in muscle tissue prompted sequencing of the entire mitochondrial genome which revealed the novel stop codon mutation m.6579G>A; p.Gly226X in MT-CO1...
February 8, 2019: Mitochondrion
Vipin Singh, Bani Jolly, Neeraj K Rajput, Sayan Pramanik, Anshu Bhardwaj
The human mitochondrion is a unique semi-autonomous organelle with a genome of its own and also requires nuclear encoded components to carry out its functions. In addition to being the powerhouse of the cell, mitochondria plays a central role in several metabolic pathways. It is therefore challenging to delineate the cause-effect relationship in context of mitochondrial dysfunction. Several studies implicate mutations in mitochondrial DNA (mtDNA) in various complex diseases. The human mitochondrial DNA (mtDNA) encodes a set of 37 genes, 13 protein coding, 22 tRNAs and two ribosomal RNAs, which are essential structural and functional components of the electron transport chain...
February 6, 2019: Mitochondrion
Yasutoshi Koga, Nataliya Povalko, Eisuke Inoue, Kazutaka Nashiki, Masashi Tanaka
Biomarkers and two clinical rating scales-the Japanese mitochondrial disease-rating scale (JMDRS) and Newcastle mitochondrial disease adult scale (NMDAS)-are clinically used when treating patients with mitochondrial disease. We explored the biomarker(s) and clinical rating scale(s) that are appropriate in preparing the protocol for a future clinical trial of sodium pyruvate (SP) therapy. A 48-week, prospective, single-centre, exploratory, clinical study enrolled 11 Japanese adult patients with genetically, biochemically, and clinically confirmed mitochondrial disease; they had intractable lactic acidosis and received SP (0...
February 6, 2019: Mitochondrion
Anuradha Sehrawat, Suman K Samanta, Eun-Ryeong Hahm, Claudette St Croix, Simon Watkins, Shivendra V Singh
Withaferin A (WA), a steroidal lactone derived from a medicinal plant (Withania somnifera), inhibits cancer development in transgenic and chemically-induced rodent models of breast cancer but the underlying mechanism is not fully grasped. We have shown previously that WA treatment causes apoptotic cell death in human breast cancer cells that is preceded by inhibition of complex III of the mitochondrial electron transport chain. This study extends these observations to now demonstrate alterations in mitochondrial dynamics in WA-induced apoptosis...
January 24, 2019: Mitochondrion
Ivo Eijkenboom, Jo M Vanoevelen, Janneke G J Hoeijmakers, Iris Wijnen, Mike Gerards, Catharina G Faber, Hubert J M Smeets
Mutations in genes involved in mitochondrial dynamics (fusion and fission) have been implicated in many peripheral neuropathies. We hypothesized that defects in these genes could result in a phenotype resembling features of small-fiber neuropathy (SFN). This was investigated in zebrafish by knocking down two genes involved in mitochondrial dynamics gdap1 (possibly fission and motility) and opa1 (fusion) using established morpholinos. Our read-outs were nerve density in the caudal fin and a behavioral response to temperature changes, both based on comparable hallmarks of SFN in patients...
January 21, 2019: Mitochondrion
Ixchel Ramírez-Camacho, Oscar Flores-Herrera, Cecilia Zazueta
Mitochondrial dysfunction, a common factor in several diseases is accompanied with reactive oxygen species (ROS) production. These molecules react with proteins and lipids at their site of generation, establishing a vicious cycle which might result in further mitochondrial injury. It is well established that mitochondrial respiratory complexes can be organized into supramolecular structures called supercomplexes (SCs) or respirasomes; yet, the physiological/pathological relevance of these structures remains unresolved...
January 18, 2019: Mitochondrion
Simon A B Knight, Heeyong Yoon, Ashutosh K Pandey, Jayashree Pain, Debkumar Pain, Andrew Dancis
Rim2 is an unusual mitochondrial carrier protein capable of transporting both iron and pyrimidine nucleotides. Here we characterize two point mutations generated in the predicted substrate-binding site, finding that they yield disparate effects on iron and pyrimidine transport. The Rim2 (E248A) mutant was deficient in mitochondrial iron transport activity. By contrast, the Rim2 (K299A) mutant specifically abrogated pyrimidine nucleotide transport and exchange, while leaving iron transport activity largely unaffected...
January 18, 2019: Mitochondrion
Aruna Pal, Abantika Pal, Samiddha Banerjee, S Batobyal, P N Chatterjee
Cytochrome B is the mitochondrial protein, which functions as part of the electron transport chain and is the main subunit of transmembrane cytochrome bc1 and b6f complexes affecting energy metabolism through oxidative phosphorylation. The present study was conducted to study the effect of mutation of Cytochrome B gene on the health condition of sheep, which the first report of association of mitochondrial gene with disease traits in livestock species. Non-synonymous substitutions (F33 L and D171N) and Indel mutations were observed for Cytochrome B gene, leading to a truncated protein, where anemia, malfunctioning of most of the vital organs as liver, kidney and mineral status was observed and debility with exercise intolerance and cardiomyopathy in extreme cases were depicted...
January 17, 2019: Mitochondrion
Dominic D Quintana, Jorge A Garcia, Saumyendra N Sarkar, Sujung Jun, Elizabeth B Engler-Chiurazzi, Ashley E Russell, John Z Cavendish, James W Simpkins
Astrocytes serve to maintain proper neuronal function and support neuronal viability, but remain largely understudied in research of cerebral ischemia. Astrocytic mitochondria are core participants in the metabolic activity of astrocytes. The objective of this study is to assess astrocyte mitochondrial competence during hypoxia and post-hypoxia reoxygenation and to determine cellular adaptive and pathological changes in the mitochondrial network. We hypothesize that during metabolic distress in astrocytes; mitochondrial networks undergo a shift in fission-fusion dynamics that results in a change in the morphometric state of the entire mitochondrial network...
December 27, 2018: Mitochondrion
Katarzyna Kuter, Łukasz Olech, Norbert A Dencher
Partial degeneration of dopaminergic neurons in the substantia nigra (SN), induces locomotor disability in animals but with time it is spontaneously compensated for by neurons surviving in the tissue by increasing their functional efficiency. Such compensation probably increases energy requirements and astrocyte support could be essential for this ability. We studied the effect of degeneration of dopaminergic neurons induced by the selective toxin 6-hydroxydopamine and/or death of 30% of astrocytes induced by chronic infusion of the glial toxin fluorocitrate on functioning of the mitochondrial electron transfer chain (ETC) complexes (Cxs) I, II, IV and their higher assembled forms, supercomplexes in the rat SN...
December 19, 2018: Mitochondrion
Justice Afrifa, Tie Zhao, Jingcui Yu
The mitochondria are defined by their unique structure and cellular functions which includes energy production, metabolic regulation, apoptosis, calcium homeostasis, cell proliferation, cell motility and transport as well as free radical generation. Recent advances geared towards enhancing the diagnostic and prognostic value of cancer patients have targeted the circulating mitochondria genome due to its specific and unique characteristics. Circulating mitochondria DNA is known to possess short length, relatively simple molecular structure and a high copy number...
December 16, 2018: Mitochondrion
Shafqat Rasul Chaudhry, Stilla Frede, Gerald Seifert, Thomas Mehari Kinfe, Mika Niemelä, Alf Lamprecht, Sajjad Muhammad
Aneurysmal subarachnoid hemorrhage (aSAH) is a highly complex disease. Majority of aSAH survivors confront post-SAH complications including cerebral vasospasm (CVS) and delayed cerebral ischemia (DCI) that mainly influence the clinical outcome. Tissue damage during early brain injury may lead to release of damage associated molecular pattern molecules (DAMPs) that may initiate and sustain inflammation during the course of aSAH through activation of pattern recognition receptors. Mitochondrial DNA (mtDNA) due to unmethylated CpG motifs acts as a DAMP via binding to toll-like receptor-9...
December 5, 2018: Mitochondrion
Krzysztof Dolowy
Ischemic preconditioning makes cells less sensitive to oxygen deprivation. A similar effect can be achieved by increasing the calcium concentration and applying potassium channel openers. A hypothetical mechanism of preconditioning is presented. In the mitochondrial matrix, there is a calcium hydroxide buffer consisting of a few insoluble calcium phosphate minerals. During ischemia, calcium ions stored in the matrix buffer start to leak out, forming an electric potential difference, while hydroxyl ions remain in the matrix, maintaining its pH and the matrix volume...
November 15, 2018: Mitochondrion
In Wook Hwang, Bit Na Kwon, Hyung Jun Kim, Seung Hun Han, Noo Ri Lee, Myung Ho Lim, Ho Jang Kwon, Han Jun Jin
Attention deficit hyperactivity disorder (ADHD) is a multifactorial disorder with multiple environmental and biological etiologies, including genetic factors. Until now, several genetic variants have been reported to be significantly associated with ADHD. Recently, the relationship between mitochondrial DNA (mtDNA) haplogroups and psychiatric disorders such as schizophrenia has also been reported. However, currently there are no reports pertaining to the genetic association between mtDNA haplogroups and ADHD...
November 10, 2018: Mitochondrion
Dorota Piekutowska-Abramczuk, Magdalena Kaliszewska, Anna Sułek, Natalia Jurkowska, Mariusz Ołtarzewski, Ewa Jabłońska, Joanna Trubicka, Aleksandra Głowacka, Elżbieta Ciara, Paweł Kowalski, Karolina Langiewicz-Wojciechowska, Marketa Tesarova, Jiri Zeman, Biruta Kierdaszuk, Dariusz Kuczyński, Dariusz Chmielewski, Edyta Szymańska, Agnieszka Bakuła, Anna Łusakowska, Marta Lipowska, Bogdan Brodacki, Joanna Pera, Małgorzata Dorobek, Małgorzata Rydzanicz, Rafał Płoski, Halina Chrzanowska Krystyna, Ewa Bartnik, Grzegorz Placha, Anna Kamińska, Anna Kostera-Pruszczyk, Małgorzata Krajewska-Walasek, Katarzyna Tońska, Ewa Pronicka
Diseases related to DNA polymerase gamma dysfunction comprise of heterogeneous clinical presentations with variable severity and age of onset. Molecular screening for the common POLG variants: p.Ala467Thr, p.Trp748Ser, p.Gly848Ser, and p.Tre251Ile has been conducted in a large population cohort (n = 3123) and in a clinically heterogeneous group of 1289 patients. Recessive pathogenic variants, including six novel ones were revealed in 22/26 patients. Infantile Alpers-Huttenlocher syndrome and adulthood ataxia spectrum were the most common found in our group...
November 10, 2018: Mitochondrion
Alyson Sujkowski, Adam N Spierer, Thiviya Rajagopalan, Brian Bazzell, Maryam Safdar, Dinko Imsirovic, Robert Arking, David Rand, Robert Wessells
Endurance exercise has received increasing attention as a broadly preventative measure against age-related disease and dysfunction. Improvement of mitochondrial quality by enhancement of mitochondrial turnover is thought to be among the important molecular mechanisms underpinning the benefits of exercise. Interactions between the mitochondrial and nuclear genomes are important components of the genetic basis for variation in longevity, fitness and the incidence of disease. Here, we examine the effects of replacing the mitochondrial genome (mtDNA) of several Drosophila strains with mtDNA from other strains, or from closely related species, on exercise performance...
November 6, 2018: Mitochondrion
Denise Mafra, Natália Alvarenga Borges, Bengt Lindholm, Peter Stenvinkel
No abstract text is available yet for this article.
November 5, 2018: Mitochondrion
Komal Panchal, Anand Krishna Tiwari
Neurodegenerative diseases (NDs) are the group of disorder that includes brain, peripheral nerves, spinal cord and results in sensory and motor neuron dysfunction. Several studies have shown that mitochondrial dynamics and their axonal transport play a central role in most common NDs such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD) and Amyotrophic Lateral Sclerosis (ALS) etc. In normal physiological condition, there is a balance between mitochondrial fission and fusion process while any alteration to these processes cause defect in ATP (Adenosine Triphosphate) biogenesis that lead to the onset of several NDs...
November 5, 2018: Mitochondrion
Leonardo de Moura Alvorcem, Renata Britto, Belisa Parmeggiani, Nícolas Manzke Glanzel, Nevton Teixeira da Rosa-Junior, Cristiane Cecatto, Larissa Daniele Bobermin, Alexandre Umpierrez Amaral, Moacir Wajner, Guilhian Leipnitz
We report here the effects of hydrogen sulfide (sulfide), that accumulates in ETHE1 deficiency, in rat cerebellum. Sulfide impaired electron transfer and oxidative phosphorylation. Sulfide also induced mitochondrial swelling, and decreased ΔΨm and calcium retention capacity in cerebellum mitochondria, which were prevented by cyclosporine A (CsA) plus ADP, and ruthenium red, suggesting mitochondrial permeability transition (mPT) induction. Melatonin (MEL) and N-ethylmaleimide also prevented sulfide-induced alterations...
November 3, 2018: Mitochondrion
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"