| Hypoxic and hypercapnic challenges unveil respiratory vulnerability of Surf1 knockout mice, an animal model of Leigh syndrome. | |
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MedLine Citation:
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PMID: 21167962 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Surf1 gene mutations were detected as a main cause for Leigh syndrome (LS), also known as infantile subacute necrotizing encephalomyelopathy. This syndrome which is commonly associated with systemic cytochrome c oxidase (COX) deficiency manifests in early childhood and has an invariable poor prognosis. Progressive disturbances of the respiratory function, for which both the metabolic condition and necrotizing brainstem lesions contribute, belong to the major symptoms of LS. A constitutive knockout (KO) mouse for Surf1 enables invasive investigations of distinct aspects of LS. In the present study the respiratory function was analyzed applying an arterially perfused brainstem preparation. Compared to wild type (WT) preparations Surf1 KO preparations had a higher baseline respiratory frequency and abnormal responses to hypoxia and hypercapnia that involved both respiratory frequency and motor nerve discharge pattern. These data suggest that COX deficiency impairs peripheral and/or central chemoreceptor function. |
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Authors:
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Georg M Stettner; Carlo Viscomi; Massimo Zeviani; Ekkehard Wilichowski; Mathias Dutschmann |
Publication Detail:
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Type: Journal Article Date: 2010-12-16 |
Journal Detail:
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Title: Mitochondrion Volume: 11 ISSN: 1872-8278 ISO Abbreviation: Mitochondrion Publication Date: 2011 May |
Date Detail:
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Created Date: 2011-04-12 Completed Date: 2011-07-25 Revised Date: 2011-11-24 |
Medline Journal Info:
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Nlm Unique ID: 100968751 Medline TA: Mitochondrion Country: Netherlands |
Other Details:
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Languages: eng Pagination: 413-20 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 Elsevier B.V. and Mitochondria Research Society. All rights reserved. |
Affiliation:
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Department of Pediatrics and Pediatric Neurology, University Medicine Göttingen, Georg August University, Robert-Koch-Str. 40, 37075 Göttingen, Germany. stettner@vet.upenn.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Anoxia / physiopathology* Chemoreceptor Cells / physiology Humans Hypercapnia / physiopathology* Leigh Disease / diagnosis*, genetics, physiopathology* Male Membrane Proteins / deficiency*, genetics, metabolism* Mice Mice, Knockout Mitochondrial Proteins / deficiency*, genetics, metabolism* Models, Animal Respiratory Rate |
| Grant Support | |
ID/Acronym/Agency:
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GGP07019//Telethon |
| Chemical | |
Reg. No./Substance:
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0/Membrane Proteins; 0/Mitochondrial Proteins; 0/Surf-1 protein |
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