A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance.

A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Research Abstract Details 

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A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Abstract Text:

N Gueven,O J Becherel,O Howe,P Chen,J-F Haince,M-E Ouellet,G G Poirier,N Waterhouse,M Fusser,B Epe,J M de Murcia,G de Murcia,C H McGowan,R Parton,C Mothersill,P Grattan-Smith,M F Lavin,

Several different autosomal recessive genetic disorders characterized by ataxia with oculomotor apraxia (AOA) have been identified with the unifying feature of defective DNA damage recognition and/or repair. We describe here the characterization of a novel form of AOA showing increased sensitivity to agents that cause single-strand breaks (SSBs) in DNA but having no gross defect in the repair of these breaks. Evidence for the presence of residual SSBs in DNA was provided by dramatically increased levels of poly (ADP-ribose)polymerase (PARP-1) auto-poly (ADP-ribosyl)ation, the detection of increased levels of reactive oxygen/nitrogen species (ROS/RNS) and oxidative damage to DNA in the patient cells. There was also evidence for oxidative damage to proteins and lipids. Although these cells were hypersensitive to DNA damaging agents, the mode of death was not by apoptosis. These cells were also resistant to TRAIL-induced death. Consistent with these observations, failure to observe a decrease in mitochondrial membrane potential, reduced cytochrome c release and defective apoptosis-inducing factor translocation to the nucleus was observed. Apoptosis resistance and PARP-1 hyperactivation were overcome by incubating the patient's cells with antioxidants. These results provide evidence for a novel form of AOA characterized by sensitivity to DNA damaging agents, oxidative stress, PARP-1 hyperactivation but resistance to apoptosis.

A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Publishing Authors By Initials

N Gueven,OJ Becherel,O Howe,P Chen,JF Haince,ME Ouellet,GG Poirier,N Waterhouse,M Fusser,B Epe,JM de Murcia,G de Murcia,CH McGowan,R Parton,C Mothersill,P Grattan-Smith,MF Lavin,

For similar inorganic chemicals: nitrogen compounds: reactive nitrogen species research abstracts see: inorganic chemicals: nitrogen compounds: reactive nitrogen species research

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A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Cell death and differentiation

VOLUME: 14

Page Numbers: 1149-61

Journal Abbreviation: Cell Death Differ.

ISSN: 1350-9047

DAY: 9

MONTH: 03

YEAR: 2007

A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Information

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LANGUAGE: eng

NlmUniqueID: 9437445

A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Keywords Mesh Terms:

KEYWORDS: Reactive Nitrogen Species

MESH TERMS: metabolism

Chemical & Substance for Abstract: A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance. Information

Substance Name: Poly(ADP-ribose) Polymerases

Registry Number: EC 2.4.2.30

Grant and Affiliation Information for A novel form of ataxia oculomotor apraxia characterized by oxidative stress and apoptosis resistance.

AFFILIATION: Department of Cancer and Cell Biology, Queensland Institute of Medical Research, Brisbane, Queensland, Australia.

Country: England

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MEDLINETA: Cell Death Differ

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