| Cell culture models to investigate the selective vulnerability of motoneuronal mitochondria to familial ALS-linked G93ASOD1. | |
| | |
MedLine Citation:
|
PMID: 16903849 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Mitochondrial damage induced by superoxide dismutase (SOD1) mutants has been proposed to have a causative role in the selective degeneration of motoneurons in amyotrophic lateral sclerosis (ALS). In order to investigate the basis of the tissue specificity of mutant SOD1 we compared the effect of the continuous expression of wild-type or mutant (G93A) human SOD1 on mitochondrial morphology in the NSC-34 motoneuronal-like, the N18TG2 neuroblastoma and the non-neuronal Madin-Darby Canine Kidney (MDCK) cell lines. Morphological alterations of mitochondria were observed in NSC-34 expressing the G93A mutant (NSC-G93A) but not the wild-type SOD1, whereas a ten-fold greater level of total expression of the mutant had no effect on mitochondria of non-motoneuronal cell lines. Fragmented network, swelling and cristae remodelling but not vacuolization of mitochondria or other intracellular organelles were observed only in NSC-G93A cells. The mitochondrial alterations were not explained by a preferential localization of the mutant within NSC-G93A mitochondria, as a higher amount of the mutant SOD1 was found in mitochondria of MDCK-G93A cells. Our results suggest that mitochondrial vulnerability of motoneurons to G93ASOD1 is recapitulated in NSC-34 cells, and that peculiar features in network dynamics may account for the selective alterations of motoneuronal mitochondria. |
| | |
Authors:
|
Andrea Raimondi; Alessandra Mangolini; Milena Rizzardini; Silvia Tartari; Silvia Massari; Caterina Bendotti; Maura Francolini; Nica Borgese; Lavinia Cantoni; Grazia Pietrini |
Related Documents
:
|
12082629 - Resistance of mitochondrial dna-deficient cells to trail: role of bax in trail-induced ... 4582949 - On the origin of mitochondrial mutants: evidence for intracellular selection of mitocho... 6188109 - Mitochondrial circular rnas are absent in sporulating cells of saccharomyces cerevisiae. 11311119 - Expression of p-170 glycoprotein sensitizes lymphoblastoid cem cells to mitochondria-me... 15475609 - Sharpening the scissors: mitochondrial fission with aid. 768759 - The induction of mutation in yeast by hydrogen peroxide. 208629 - Lipoprotein and cholesterol metabolism in rabbit arterial endothelial cells in culture. 18504979 - Whole cell electrochemistry of electricity-producing microorganisms evidence an adaptat... 15347969 - In situ localization of apoptotic changes in the interface membrane of aseptically loos... |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
|
Title: The European journal of neuroscience Volume: 24 ISSN: 0953-816X ISO Abbreviation: Eur. J. Neurosci. Publication Date: 2006 Jul |
Date Detail:
|
Created Date: 2006-08-14 Completed Date: 2006-10-03 Revised Date: 2006-11-15 |
Medline Journal Info:
|
Nlm Unique ID: 8918110 Medline TA: Eur J Neurosci Country: France |
Other Details:
|
Languages: eng Pagination: 387-99 Citation Subset: IM |
Affiliation:
|
Department of Pharmacology, School of Medicine, Center of Excellence on Neurodegenerative Diseases, University of Milano, Consiglio Nazionale delle Ricerche, CNR, Institute of Neuroscience, Milano, Italy. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Amyotrophic Lateral Sclerosis
/
enzymology*,
genetics*,
physiopathology Animals Cell Line, Tumor Cell Respiration / genetics Dogs Gene Expression Regulation, Enzymologic / genetics Genetic Predisposition to Disease / genetics* Humans Mice Microscopy, Electron, Transmission Mitochondria / enzymology*, genetics, pathology Mitochondrial Membranes / enzymology, pathology Motor Neurons / enzymology*, pathology Mutation / genetics Superoxide Dismutase / genetics* |
| Chemical | |
Reg. No./Substance:
|
EC 1.15.1.-/superoxide dismutase 1; EC 1.15.1.1/Superoxide Dismutase |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Astroglia up-regulate transcription and secretion of 'readthrough' acetylcholinesterase following ox...
Next Document: Inhibition of constitutive inward rectifier currents in cerebellar granule cells by pharmacological ...