Masci 2008 Biochim Biophys Acta

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.

» PMID: 18047827

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. • Keywords: Nitrosative stress, Bioenergetics, Cytochrome c oxidase, Genetic disease, Reaction mechanism, Respiratory chain

• O2k-Network Lab: IT_Roma_Sarti P

Labels:

Stress:RONS; Oxidative Stress"RONS; Oxidative Stress" is not in the list (Cell death, Cryopreservation, Ischemia-reperfusion, Permeability transition, Oxidative stress;RONS, Temperature, Hypoxia, Mitochondrial disease) of allowed values for the "Stress" property.  Organism: Human  Tissue;cell: Nervous system  Preparation: Intact cells  Enzyme: Complex IV; Cytochrome c Oxidase"Complex IV; Cytochrome c Oxidase" is not in the list (Adenine nucleotide translocase, Complex I, Complex II;succinate dehydrogenase, Complex III, Complex IV;cytochrome c oxidase, Complex V;ATP synthase, Inner mt-membrane transporter, Marker enzyme, Supercomplex, TCA cycle and matrix dehydrogenases, ...) of allowed values for the "Enzyme" property.  Regulation: Ion&substrate transport"Ion&substrate transport" is not in the list (Aerobic glycolysis, ADP, ATP, ATP production, AMP, Calcium, Coupling efficiency;uncoupling, Cyt c, Flux control, Inhibitor, ...) of allowed values for the "Respiration and regulation" property., Redox state  Coupling state: OXPHOS 

HRR: Oxygraph-2k