scholarly journals The Common miRNA Signatures Associated with Mitochondrial Dysfunction in Different Muscular Dystrophies

2020 ◽  
Vol 190 (10) ◽  
pp. 2136-2145
Author(s):  
Evrim Aksu-Menges ◽  
Yeliz Z. Akkaya-Ulum ◽  
Didem Dayangac-Erden ◽  
Banu Balci-Peynircioglu ◽  
Ayse Yuzbasioglu ◽  
...  
Keyword(s):  
Mitochondrial Dysfunction ◽  
Muscular Dystrophies ◽  
The Common

scholarly journals Dlk1-Dio3 cluster miRNAs regulate mitochondrial functions in Duchenne muscular dystrophy

2021 ◽  
Author(s):  
Ai VU Hong ◽  
Nathalie Bourg ◽  
Peggy Sanatine ◽  
Jerome Poupiot ◽  
Karine Charton ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Mitochondrial Dysfunction ◽  
Muscular Dystrophies ◽  
Mirna Cluster ◽  
Biological Functions ◽  
Dystrophic Muscle ◽  
Mitochondrial Functions ◽  
Mirnas Expression

Background: Duchenne Muscular Dystrophy (DMD) is a severe muscle disease caused by impaired expression of dystrophin. While mitochondrial dysfunction is thought to play an important role in DMD, the mechanism of this dysfunction remains to be clarified. We recently identified in DMD and in other muscular dystrophies the upregulation of a large number of the Dlk1-Dio3 clustered miRNAs (DD-miRNAs), in both the muscle and the serum. The objective of the present study was to define the biological functions of DD-miRNAs in skeletal muscle, particularly in the context of muscular dystrophy. Methods: DD-miRNAs expression pattern was characterized in vitro and in vivo, in normal and dystrophic situations. Epigenomic characterization was performed, to elucidate the molecular control of DD-miRNAs dysregulation. The biological effect of muscle DD-miRNAs dysregulation was investigated by an in vivo simultaneous overexpression of 14 DD-miRNAs in the wild-type muscle, together with CRISPR-Cas9-based knockdown of the entire DD-miRNA cluster in an iPS-derived myotubes. Omics data and bioinformatics tools were used for the prediction of DD-miRNAs biological functions, and functional characterization of mitochondrial pathways was performed. Results: We found that DD-miRNAs dysregulation is not specific to DMD since observed in mouse models for other muscular dystrophies. We showed that DD-miRNAs expression in mdx, is reduced in satellite cells, but highly upregulated in regenerating myofibers, suggesting a myofibers origin of DD6miRNA upregulation in muscular dystrophy in both muscles and serum. We demonstrated that upregulation of DD-miRNAs in the dystrophic muscle is controlled epigenetically by DNA and histone methylation (p<0.0001 and p=0.001, respectively) at the Intergenic Differentially Methylated Region (IG-DMR) of Dlk1-Dio3 locus. Transcriptomic analysis revealed a substantial overlap between the dystrophic muscle of the mdx mouse and the normal muscle that overexpressed 14 DD-miRNAs. Bioinformatics analysis predicted that DD-miRNAs could regulate mitochondrial functions. The ectopic overexpression of 14 DD-miRNAs, in the healthy muscle, resulted in a drastic downregulation of mitochondrial oxidative phosphorylation (OxPhos) (NES=-2.8, p=8.7E-17), similarly to the level in dystrophic muscles of mdx mice and DMD patients (NES=-2.88, p=7.7E-28). Knocking down the entire DD-miRNA cluster in iPS-derived myotubes resulted in increased mitochondrial OxPhos expression and activities. Conclusions: The present study provides evidence for the modulation of mitochondrial activity in the dystrophic muscle by the upregulated DD-miRNAs and supports an updated model for mitochondrial dysfunction in DMD. The regulation of mitochondrial OxPhos by DD-miRNAs may have a broader impact beyond DMD in physiological and pathological situations of muscle adaptation and regeneration.

Nascent Protein Labeling Strategy Disclosed Mitochondrial Proteomic Response in Punicalagin Intervened Insulin Resistance of HepG2 Cells

2022 ◽  
Author(s):  
Zhengyi Zhang ◽  
Mengqi Zeng ◽  
Xiao Han ◽  
Zhanwu Hou ◽  
Zhen Wang ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Mitochondrial Dysfunction ◽  
Hepg2 Cells ◽  
Metabolic Diseases ◽  
Protein Labeling ◽  
Obesity And Diabetes ◽  
The Common ◽  
Proteomic Response

Insulin Resistance (IR), as the common pathophysiological basis, is closely related to a variety of metabolic diseases, such as obesity and diabetes. IR is often accompanied with mitochondrial dysfunction which...

Catalogue reduction techniques

1978 ◽  
Vol 48 ◽  
pp. 389-390 ◽  
Author(s):  
Chr. de Vegt
Keyword(s):  
Adjustment Process ◽  
Proper Motions ◽  
Measuring Process ◽  
Aerial Photogrammetry ◽  
Reduction Techniques ◽  
Observational Technique ◽  
The Common ◽  
Astrometric Data

AbstractReduction techniques as applied to astrometric data material tend to split up traditionally into at least two different classes according to the observational technique used, namely transit circle observations and photographic observations. Although it is not realized fully in practice at present, the application of a blockadjustment technique for all kind of catalogue reductions is suggested. The term blockadjustment shall denote in this context the common adjustment of the principal unknowns which are the positions, proper motions and certain reduction parameters modelling the systematic properties of the observational process. Especially for old epoch catalogue data we frequently meet the situation that no independent detailed information on the telescope properties and other instrumental parameters, describing for example the measuring process, is available from special calibration observations or measurements; therefore the adjustment process should be highly self-calibrating, that means: all necessary information has to be extracted from the catalogue data themselves. Successful applications of this concept have been made already in the field of aerial photogrammetry.

Renilla Bioluminescence: A Morphologic Approach to the Location of Photocytes

1974 ◽  
Vol 32 ◽  
pp. 208-209
Author(s):  
Ben O. Spurlock ◽  
Milton J. Cormier
Keyword(s):  
Excited State ◽  
Ground State ◽  
Molecular Basis ◽  
Light Emission ◽  
Chemical Basis ◽  
Electronic Excited State ◽  
Colonial Form ◽  
The Common ◽  
Eighteenth And Nineteenth Centuries ◽  
Catalyzed Oxidation

The phenomenon of bioluminescence has fascinated layman and scientist alike for many centuries. During the eighteenth and nineteenth centuries a number of observations were reported on the physiology of bioluminescence in Renilla, the common sea pansy. More recently biochemists have directed their attention to the molecular basis of luminosity in this colonial form. These studies have centered primarily on defining the chemical basis for bioluminescence and its control. It is now established that bioluminescence in Renilla arises due to the luciferase-catalyzed oxidation of luciferin. This results in the creation of a product (oxyluciferin) in an electronic excited state. The transition of oxyluciferin from its excited state to the ground state leads to light emission.

Mammalian Bone Marrow Acetylcholinesterase

1982 ◽  
Vol 40 ◽  
pp. 44-45
Author(s):  
Ezzatollah Keyhani
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
Common Property ◽  
Extracellular Medium ◽  
The Central Nervous System ◽  
The Common ◽  
Mammalian Bone

Acetylcholinesterase (EC 3.1.1.7) (ACHE) has been localized at cholinergic junctions both in the central nervous system and at the periphery and it functions in neurotransmission. ACHE was also found in other tissues without involvement in neurotransmission, but exhibiting the common property of transporting water and ions. This communication describes intracellular ACHE in mammalian bone marrow and its secretion into the extracellular medium.

Correlation analysis of radiation damage

1978 ◽  
Vol 36 (1) ◽  
pp. 214-215
Author(s):  
R. Hegerl ◽  
A. Feltynowski ◽  
B. Grill
Keyword(s):  
Electron Microscopy ◽  
Independent Random Variables ◽  
Optical Parameters ◽  
Bright Field Image ◽  
Bright Field ◽  
Expectation Value ◽  
All Optical ◽  
Image Element ◽  
Stochastic Series ◽  
The Common

Till now correlation functions have been used in electron microscopy for two purposes: a) to find the common origin of two micrographs representing the same object, b) to check the optical parameters e. g. the focus. There is a third possibility of application, if all optical parameters are constant during a series of exposures. In this case all differences between the micrographs can only be caused by different noise distributions and by modifications of the object induced by radiation.Because of the electron noise, a discrete bright field image can be considered as a stochastic series Pm,where i denotes the number of the image and m (m = 1,.., M) the image element. Assuming a stable object, the expectation value of Pm would be Ηm for all images. The electron noise can be introduced by addition of stationary, mutual independent random variables nm with zero expectation and the variance. It is possible to treat the modifications of the object as a noise, too.

The Red Neuronal Pigment in the Nervous System of the Mussel, Mytilus Edulis: Localization and Its Effect on Lateral Ciliary Activity with Spectral and Analytical Changes

1981 ◽  
Vol 39 ◽  
pp. 494-495
Author(s):  
Anthony A. Paparo ◽  
Judith A. Murphy
Keyword(s):  
Nervous System ◽  
Mytilus Edulis ◽  
Neuronal Cell ◽  
Ciliary Activity ◽  
Neuronal Cell Bodies ◽  
The Central Nervous System ◽  
Intracellular Organelles ◽  
The Common ◽  
The Individual ◽  
Cryostat Sections

The purpose of this study was to localize the red neuronal pigment in Mytilus edulis and examine its role in the control of lateral ciliary activity in the gill. The visceral ganglia (Vg) in the central nervous system show an over al red pigmentation. Most red pigments examined in squash preps and cryostat sec tions were localized in the neuronal cell bodies and proximal axon regions. Unstained cryostat sections showed highly localized patches of this pigment scattered throughout the cells in the form of dense granular masses about 5-7 um in diameter, with the individual granules ranging from 0.6-1.3 um in diame ter. Tissue stained with Gomori's method for Fe showed bright blue granular masses of about the same size and structure as previously seen in unstained cryostat sections.Thick section microanalysis (Fig.l) confirmed both the localization and presence of Fe in the nerve cell. These nerve cells of the Vg share with other pigmented photosensitive cells the common cytostructural feature of localization of absorbing molecules in intracellular organelles where they are tightly ordered in fine substructures.

Sign in / Sign up

Export Citation Format

Share Document