scholarly journals Role of myelin sheath energy metabolism in neurodegenerative diseases

2015 ◽  
Vol 10 (10) ◽  
pp. 1570 ◽  
Author(s):  
Silvia Ravera ◽  
Isabella Panfoli
Keyword(s):  
Energy Metabolism ◽  
Neurodegenerative Diseases ◽  
Myelin Sheath

scholarly journals MNRR1, a Biorganellar Regulator of Mitochondria

2017 ◽  
Vol 2017 ◽  
pp. 1-12 ◽  
Author(s):  
Lawrence I. Grossman ◽  
Neeraja Purandare ◽  
Rooshan Arshad ◽  
Stephanie Gladyck ◽  
Mallika Somayajulu ◽  
...  
Keyword(s):  
Energy Metabolism ◽  
Neurodegenerative Diseases ◽  
Family Members ◽  
Unusual Property ◽  
Promoter Element ◽  
Low Oxygen ◽  
Oxidative Stresses ◽  
Graded Response ◽  
Oxygen Tensions

The central role of energy metabolism in cellular activities is becoming widely recognized. However, there are many gaps in our knowledge of the mechanisms by which mitochondria evaluate their status and call upon the nucleus to make adjustments. Recently, a protein family consisting of twin CX9C proteins has been shown to play a role in human pathophysiology. We focus here on two family members, the isoforms CHCHD2 (renamed MNRR1) and CHCHD10. The better studied isoform, MNRR1, has the unusual property of functioning in both the mitochondria and the nucleus and of having a different function in each. In the mitochondria, it functions by binding to cytochromecoxidase (COX), which stimulates respiration. Its binding to COX is promoted by tyrosine-99 phosphorylation, carried out by ABL2 kinase (ARG). In the nucleus, MNRR1 binds to a novel promoter element inCOX4I2and itself, increasing transcription at 4% oxygen. We discuss mutations in both MNRR1 and CHCHD10 found in a number of chronic, mostly neurodegenerative, diseases. Finally, we propose a model of a graded response to hypoxic and oxidative stresses, mediated under different oxygen tensions by CHCHD10, MNRR1, and HIF1, which operate at intermediate and very low oxygen concentrations, respectively.

scholarly journals Oligodendrocytes: Morphology, functions and involvement in neurodegenerative diseases

2019 ◽  
Vol 75 (05) ◽  
pp. 6258-2019
Author(s):  
IZABELA KRAWCZYK-MARĆ ◽  
AGATA WAWRZYNIAK ◽  
IWONA ŁUSZCZEWSKA-SIERAKOWSKA ◽  
MAREK BABICZ ◽  
STANISŁAW ORKISZ
Keyword(s):  
Neurodegenerative Diseases ◽  
Glial Cells ◽  
Myelin Sheath ◽  
Action Potentials ◽  
Neurological Diseases ◽  
Radical Change ◽  
Differentiation Process ◽  
Myelin Sheaths ◽  
The Central Nervous System

Oligodendrocytes (OLs) are myelinating cells of the central nervous system (CNS). They are a highly specialized type of glial cell in the CNS of vertebrates, which guarantee the transmission of action potentials over long distances by producing a myelin sheath that wraps adjacent axons. Although they are often credited merely with participation in myelination, recent research has led to a radical change in the understanding the role of these glial cells. OLs are currently understood to be plastic and adaptive cells, capable of responding quickly to changes taking place in the spatial neuronal network in the CNS. Due to their complex differentiation process and their physiology, OLs are among the most sensitive cells in the CNS. Finding answers about their interactions with other types of glial cells may result in benefits in the form of neuroprotection and axon plasticity. Damage to OLs and the myelin sheath is one of processes contributing to the development of crippling neurological diseases, although the role of these cells in neurodegeneration remains controversial. This article not only presents OLs as cells whose ultimate goal is to produce myelin sheaths, but also discusses their involvement in neurodegenerative diseases.

scholarly journals ER-mitochondria cross-talk is regulated by the Ca2+ sensor NCS1 and is impaired in Wolfram syndrome

2018 ◽  
Vol 11 (553) ◽  
pp. eaaq1380 ◽  
Author(s):  
Claire Angebault ◽  
Jérémy Fauconnier ◽  
Simone Patergnani ◽  
Jennifer Rieusset ◽  
Alberto Danese ◽  
...  
Keyword(s):  
Endoplasmic Reticulum ◽  
Energy Metabolism ◽  
Neurodegenerative Diseases ◽  
Cell Survival ◽  
Cross Talk ◽  
Wolfram Syndrome ◽  
Calcium Sensor ◽  
Inositol 1,4,5 Trisphosphate ◽  
Trisphosphate Receptor

Communication between the endoplasmic reticulum (ER) and mitochondria plays a pivotal role in Ca2+ signaling, energy metabolism, and cell survival. Dysfunction in this cross-talk leads to metabolic and neurodegenerative diseases. Wolfram syndrome is a fatal neurodegenerative disease caused by mutations in the ER-resident protein WFS1. Here, we showed that WFS1 formed a complex with neuronal calcium sensor 1 (NCS1) and inositol 1,4,5-trisphosphate receptor (IP3R) to promote Ca2+ transfer between the ER and mitochondria. In addition, we found that NCS1 abundance was reduced in WFS1-null patient fibroblasts, which showed reduced ER-mitochondria interactions and Ca2+ exchange. Moreover, in WFS1-deficient cells, NCS1 overexpression not only restored ER-mitochondria interactions and Ca2+ transfer but also rescued mitochondrial dysfunction. Our results describe a key role of NCS1 in ER-mitochondria cross-talk, uncover a pathogenic mechanism for Wolfram syndrome, and potentially reveal insights into the pathogenesis of other neurodegenerative diseases.

Mitochondrial matrix calcium ions regulate energy production in myocardium: A cytochemical study

1990 ◽  
Vol 48 (2) ◽  
pp. 166-167
Author(s):  
W.A. Jacob ◽  
R. Hertsens ◽  
A. Van Bogaert ◽  
M. De Smet
Keyword(s):  
Energy Metabolism ◽  
Calcium Ions ◽  
Energy Production ◽  
Regulation Of Respiration ◽  
Free Calcium ◽  
Mitochondrial Matrix ◽  
Cytochemical Study ◽  
Nmr Techniques

In the past most studies of the control of energy metabolism focus on the role of the phosphorylation potential ATP/ADP.Pi on the regulation of respiration. Studies using NMR techniques have demonstrated that the concentrations of these compounds for oxidation phosphorylation do not change appreciably throughout the cardiac cycle and during increases in cardiac work. Hence regulation of energy production by calcium ions, present in the mitochondrial matrix, has been the object of a number of recent studies.Three exclusively intramitochondnal dehydrogenases are key enzymes for the regulation of oxidative metabolism. They are activated by calcium ions in the low micromolar range. Since, however, earlier estimates of the intramitochondnal calcium, based on equilibrium thermodynamic considerations, were in the millimolar range, a physiological correlation was not evident. The introduction of calcium-sensitive probes fura-2 and indo-1 made monitoring of free calcium during changing energy metabolism possible. These studies were performed on isolated mitochondria and extrapolation to the in vivo situation is more or less speculative.

Role of the Structural Characteristics of Dendrimers in the Manifestation of the Antiamyloid Properties

INEOS OPEN ◽  
2020 ◽  
Vol 3 ◽  
Author(s):  
S. A. Sorokina ◽  
◽  
Yu. Yu. Stroilova ◽  
V. I. Muronets ◽  
Z. B. Shifrina ◽  
...  
Keyword(s):  
Neurodegenerative Diseases ◽  
Structural Characteristics ◽  
Short Review ◽  
External Factors ◽  
Amyloid Aggregation ◽  
Amyloid Proteins ◽  
Molecular Parameters ◽  
Amyloid Aggregates ◽  
Aggregation Processes

Among the compounds able to efficiently inhibit the amyloid aggregation of proteins and decompose the amyloid aggregates that cause neurodegenerative diseases, of particular interest are dendrimers, which represent individual macromolecules with the hypercrosslinked architectures and given molecular parameters. This short review outlines the peculiarities of the antiamyloid activity of dendrimers and discusses the effect of dendrimer structures and external factors on their antiamyloid properties. The potential of application of dendrimers in further investigations on the aggregation processes of amyloid proteins as the compounds that exhibit the remarkable antiamyloid activity is evaluated.

Role of GPRC6A in Regulating Hepatic Energy Metabolism

2019 ◽  
Author(s):  
Min Pi ◽  
Fuyi Xu ◽  
Ruisong Ye ◽  
Satoru K. Nishimoto ◽  
Robert A. Kesterson ◽  
...  
Keyword(s):  
Energy Metabolism ◽  
Hepatic Energy Metabolism

Involvement of Heme Oxygenase-1 in Neuropsychiatric and Neurodegenerative Diseases

2018 ◽  
Vol 24 (20) ◽  
pp. 2283-2302 ◽  
Author(s):  
Vivian B. Neis ◽  
Priscila B. Rosa ◽  
Morgana Moretti ◽  
Ana Lucia S. Rodrigues
Keyword(s):  
Neurodegenerative Diseases ◽  
Heme Oxygenase ◽  
Therapeutic Potential ◽  
Redox Homeostasis ◽  
Antioxidant Defenses ◽  
Heme Oxygenase 1 ◽  
Physiological Conditions ◽  
And Oxidative Stress ◽  
Defensive Mechanism

Heme oxygenase (HO) family catalyzes the conversion of heme into free iron, carbon monoxide and biliverdin. It possesses two well-characterized isoforms: HO-1 and HO-2. Under brain physiological conditions, the expression of HO-2 is constitutive, abundant and ubiquitous, whereas HO-1 mRNA and protein are restricted to small populations of neurons and neuroglia. HO-1 is an inducible enzyme that has been shown to participate as an essential defensive mechanism for neurons exposed to oxidant challenges, being related to antioxidant defenses in certain neuropathological conditions. Considering that neurodegenerative diseases (Alzheimer’s Disease (AD), Parkinson’s Disease (PD) and Multiple Sclerosis (MS)) and neuropsychiatric disorders (depression, anxiety, Bipolar Disorder (BD) and schizophrenia) are associated with increased inflammatory markers, impaired redox homeostasis and oxidative stress, conditions that may be associated with alterations in HO-levels/activity, the purpose of this review is to present evidence on the possible role of HO-1 in these Central Nervous System (CNS) diseases. In addition, the possible therapeutic potential of targeting brain HO-1 is explored in this review.

Role of paraoxonase 1 (PON1) in organophosphate metabolism: Implications in neurodegenerative diseases

2011 ◽  
Vol 256 (3) ◽  
pp. 418-424 ◽  
Author(s):  
Vasilis P. Androutsopoulos ◽  
Konstantinos Kanavouras ◽  
Aristidis M. Tsatsakis
Keyword(s):  
Neurodegenerative Diseases ◽  
Paraoxonase 1
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