Masci 2008 Biochim Biophys Acta

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Masci A, Mastronicola D, Arese M, Piane M, De Amicis A, Blanck TJ, Chessa L, Sarti P (2008) Control of cell respiration by nitric oxide in Ataxia Telangiectasia lymphoblastoid cells. Biochim Biophys Acta 1777:66-73.

Β» PMID: 18047827 Open Access

Masci A, Mastronicola D, Arese M, Piane M, De Amicis A, Blanck TJ, Chessa L, Sarti P (2008) Biochim Biophys Acta

Abstract: Ataxia Telangiectasia (AT) patients are particularly sensitive to oxidative–nitrosative stress. Nitric oxide (NO) controls mitochondrial respiration via the reversible inhibition of complex IV. The mitochondrial response to NO of AT lymphoblastoid cells was investigated. Cells isolated from three patients and three intrafamilial healthy controls were selected showing within each group a normal diploid karyotype and homogeneous telomere length. Different complex IV NO-inhibition patterns were induced by varying the electron flux through the respiratory chain, using exogenous cell membrane permeable electron donors. Under conditions of high electron flux the mitochondrial NO inhibition of respiration was greater in AT than in control cells (P ≀ 0.05). This property appears peculiar to AT, and correlates well to the higher concentration of cytochrome c detected in the AT cells. This finding is discussed on the basis of the proposed mechanism of reaction of NO with complex IV. It is suggested that the peculiar response of AT mitochondria to NO stress may be relevant to the mitochondrial metabolism of AT patients. β€’ Keywords: Nitrosative stress, Bioenergetics, Cytochrome c oxidase, Genetic disease, Reaction mechanism, Respiratory chain

β€’ O2k-Network Lab: IT Rome Sarti P


Stress:Oxidative stress;RONS  Organism: Human  Tissue;cell: Nervous system  Preparation: Intact cells  Enzyme: Complex IV;cytochrome c oxidase  Regulation: Ion;substrate transport, Redox state  Coupling state: OXPHOS 

HRR: Oxygraph-2k, NO 

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