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Difference between revisions of "Ehinger 2015 J Neurol"

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{{Publication
{{Publication
|title=Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients (2015) Ehinger JK1, Morota S, Hansson MJ, Paul G, Elmér E. J Neurol 262:1493-503.  
|title=Ehinger JK, Morota S, Hansson MJ, Paul G, Elmér E (2015) Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients. J Neurol 262:1493-503.
|info=[http://www.ncbi.nlm.nih.gov/pubmed/25893255 PMID: 25893255]
|info=[http://www.ncbi.nlm.nih.gov/pubmed/25893255 PMID: 25893255]
|authors=Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients
|authors=Ehinger JK, Morota S, Hansson MJ, Paul G, Elmer E
|year=2015
|year=2015
|journal=J Neurol
|journal=J Neurol
|abstract=Mitochondrial dysfunction is implicated in amyotrophic lateral sclerosis, where the progressive degeneration of motor neurons results in muscle atrophy, paralysis and death. Abnormalities in both central nervous system and muscle mitochondria have previously been demonstrated in patient samples, indicating systemic disease. In this case-control study, venous blood samples were acquired from 24 amyotrophic lateral sclerosis patients and 21 age-matched controls. Platelets and peripheral blood mononuclear cells were isolated and mitochondrial oxygen consumption measured in intact and permeabilized cells with additions of mitochondrial substrates, inhibitors and titration of an uncoupler. Respiratory values were normalized to cell count and for two markers of cellular mitochondrial content, citrate synthase activity and mitochondrial DNA, respectively. Mitochondrial function was correlated with clinical staging of disease severity. Complex IV (cytochrome c-oxidase)-activity normalized to mitochondrial content was decreased in platelets from amyotrophic lateral sclerosis patients both when normalized to citrate synthase activity and mitochondrial DNA copy number. In mononuclear cells, complex IV-activity was decreased when normalized to citrate synthase activity. Mitochondrial content was increased in amyotrophic lateral sclerosis patient platelets. In mononuclear cells, complex I activity declined and mitochondrial content increased progressively with advancing disease stage. The findings are, however, based on small subsets of patients and need to be confirmed. We conclude that when normalized to mitochondria-specific content, complex IV-activity is reduced in blood cells from amyotrophic lateral sclerosis patients and that there is an apparent compensatory increase in cellular mitochondrial content. This supports systemic involvement in amyotrophic lateral sclerosis and suggests further study of mitochondrial function in blood cells as a future biomarker for the disease.
|abstract=Mitochondrial dysfunction is implicated in amyotrophic lateral sclerosis, where the progressive degeneration of motor neurons results in muscle atrophy, paralysis and death. Abnormalities in both central nervous system and muscle mitochondria have previously been demonstrated in patient samples, indicating systemic disease. In this case-control study, venous blood samples were acquired from 24 amyotrophic lateral sclerosis patients and 21 age-matched controls. Platelets and peripheral blood mononuclear cells were isolated and mitochondrial oxygen consumption measured in intact and permeabilized cells with additions of mitochondrial substrates, inhibitors and titration of an uncoupler. Respiratory values were normalized to cell count and for two markers of cellular mitochondrial content, citrate synthase activity and mitochondrial DNA, respectively. Mitochondrial function was correlated with clinical staging of disease severity. Complex IV (cytochrome c-oxidase)-activity normalized to mitochondrial content was decreased in platelets from amyotrophic lateral sclerosis patients both when normalized to citrate synthase activity and mitochondrial DNA copy number. In mononuclear cells, complex IV-activity was decreased when normalized to citrate synthase activity. Mitochondrial content was increased in amyotrophic lateral sclerosis patient platelets. In mononuclear cells, complex I activity declined and mitochondrial content increased progressively with advancing disease stage. The findings are, however, based on small subsets of patients and need to be confirmed. We conclude that when normalized to mitochondria-specific content, complex IV-activity is reduced in blood cells from amyotrophic lateral sclerosis patients and that there is an apparent compensatory increase in cellular mitochondrial content. This supports systemic involvement in amyotrophic lateral sclerosis and suggests further study of mitochondrial function in blood cells as a future biomarker for the disease.
|keywords=Amyotrophic lateral sclerosis, Mitochondria, Biomarkers, Mitochondrial complex IV deficiency, Motor neurons
|keywords=Amyotrophic lateral sclerosis, Mitochondria, Biomarkers, Mitochondrial complex IV deficiency, Motor neurons, PBMC
|mipnetlab=SE Lund Elmer E
|mipnetlab=SE Lund Elmer E, JP Tokyo Uchino H
}}
}}
== Cited by ==
{{Template:Cited by Gnaiger 2020 BEC MitoPhysiology}}
{{Labeling
{{Labeling
|area=Respiration, mtDNA;mt-genetics, Patients
|area=Respiration, mtDNA;mt-genetics, mt-Medicine, Patients
|diseases=Neurodegenerative, Other
|organism=Human
|organism=Human
|tissues=Blood cells
|tissues=Blood cells, Lymphocyte, Platelet
|preparations=Intact cells, Permeabilized cells
|preparations=Permeabilized cells, Intact cells
|diseases=Neurodegenerative
|couplingstates=LEAK, ROUTINE, OXPHOS, ET
|couplingstates=LEAK, ROUTINE, OXPHOS, ETS
|pathways=N, S, CIV, NS, ROX
|substratestates=CI, CIII, CI&II, ROX
|instruments=Oxygraph-2k
|instruments=Oxygraph-2k
|additional=Labels
|additional=JP, SE, MitoEAGLE blood cells data, BEC 2020.1
}}
}}

Latest revision as of 17:28, 16 January 2021

Publications in the MiPMap
Ehinger JK, Morota S, Hansson MJ, Paul G, Elmér E (2015) Mitochondrial dysfunction in blood cells from amyotrophic lateral sclerosis patients. J Neurol 262:1493-503.

» PMID: 25893255

Ehinger JK, Morota S, Hansson MJ, Paul G, Elmer E (2015) J Neurol

Abstract: Mitochondrial dysfunction is implicated in amyotrophic lateral sclerosis, where the progressive degeneration of motor neurons results in muscle atrophy, paralysis and death. Abnormalities in both central nervous system and muscle mitochondria have previously been demonstrated in patient samples, indicating systemic disease. In this case-control study, venous blood samples were acquired from 24 amyotrophic lateral sclerosis patients and 21 age-matched controls. Platelets and peripheral blood mononuclear cells were isolated and mitochondrial oxygen consumption measured in intact and permeabilized cells with additions of mitochondrial substrates, inhibitors and titration of an uncoupler. Respiratory values were normalized to cell count and for two markers of cellular mitochondrial content, citrate synthase activity and mitochondrial DNA, respectively. Mitochondrial function was correlated with clinical staging of disease severity. Complex IV (cytochrome c-oxidase)-activity normalized to mitochondrial content was decreased in platelets from amyotrophic lateral sclerosis patients both when normalized to citrate synthase activity and mitochondrial DNA copy number. In mononuclear cells, complex IV-activity was decreased when normalized to citrate synthase activity. Mitochondrial content was increased in amyotrophic lateral sclerosis patient platelets. In mononuclear cells, complex I activity declined and mitochondrial content increased progressively with advancing disease stage. The findings are, however, based on small subsets of patients and need to be confirmed. We conclude that when normalized to mitochondria-specific content, complex IV-activity is reduced in blood cells from amyotrophic lateral sclerosis patients and that there is an apparent compensatory increase in cellular mitochondrial content. This supports systemic involvement in amyotrophic lateral sclerosis and suggests further study of mitochondrial function in blood cells as a future biomarker for the disease. Keywords: Amyotrophic lateral sclerosis, Mitochondria, Biomarkers, Mitochondrial complex IV deficiency, Motor neurons, PBMC

O2k-Network Lab: SE Lund Elmer E, JP Tokyo Uchino H

Cited by

Gnaiger Erich et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology. Bioenerg Commun 2020.1.
Gnaiger E et al ― MitoEAGLE Task Group (2020) Mitochondrial physiology. Bioenerg Commun 2020.1. doi:10.26124/bec:2020-0001.v1.



Labels: MiParea: Respiration, mtDNA;mt-genetics, mt-Medicine, Patients  Pathology: Neurodegenerative, Other 

Organism: Human  Tissue;cell: Blood cells, Lymphocyte, Platelet  Preparation: Permeabilized cells, Intact cells 


Coupling state: LEAK, ROUTINE, OXPHOS, ET  Pathway: N, S, CIV, NS, ROX  HRR: Oxygraph-2k 

JP, SE, MitoEAGLE blood cells data, BEC 2020.1