Drilling for Energy in Mitochondrial Disease

Haller, Ronald G.; Vissing, John

doi:10.1001/archneurol.2009.155

Cited by 5 publications

(3 citation statements)

References 17 publications

(16 reference statements)

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“…Another source of anerobic ATP production has been exploited recently to increase substrate level phosphorylation: The succinyl coenzyme A synthase in the TCA cycle catalyzes substratelevel phosphorylation of ADP or GDP. In a novel approach, exogenous substrates capable of stimulating the Krebs cycle flux were used to activate the enzymes [Haller and Vissing, 2009]. At the same time excess of reduced pyrimidine nucleotides (nicotinamide adenine dinucleotide [NADH]) were removed by reversal of the normal flux of the Krebs cycle with oxaloacetate converted to malate to avoid accumulation of succinyl-CoA [Sgarbi et al, 2009].…”

Section: Optimizing Substrate Level Phosphorylationmentioning

confidence: 99%

Emerging therapeutic approaches to mitochondrial diseases

Wenz

Williams

Bacman

et al. 2010

Dev Disabil Res Revs

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Mitochondrial diseases are very heterogeneous and can affect different tissues and organs. Moreover, they can be caused by genetic defects in either nuclear or mitochondrial DNA as well as by environmental factors. All of these factors have made the development of therapies difficult. In this review article, we will discuss emerging approaches to the therapy of mitochondrial disorders, some of which are targeted to specific conditions whereas others may be applicable to a more diverse group of patients.

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Section: Optimizing Substrate Level Phosphorylationmentioning

confidence: 99%

Emerging therapeutic approaches to mitochondrial diseases

Wenz

Williams

Bacman

et al. 2010

Dev Disabil Res Revs

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“…ETC enhancement was initially attempted in encephalomyopathy patients [3,54]. Reported outcomes vary.…”

Section: Mitochondrial Medicinementioning

confidence: 99%

Mitochondrial Medicine and the Neurodegenerative Mitochondriopathies

Swerdlow

2009

Pharmaceuticals

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Neurodegenerative diseases are a common late-life scourge for which disease-modifying treatments are sorely needed. Mitochondrial perturbation is commonly observed in these diseases, so pursuing treatment development strategies that target mitochondria or processes affected by mitochondria seems reasonable. This review discusses the rationale underlying past and current efforts to treat neurodegenerative diseases using mitochondrial medicine, and tries to predict how future efforts might proceed.

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“…The first part of this review discusses evidence that argues mtDNA is relevant to various neurodegenerative diseases. The second part discusses therapeutic options from the perspective of an evolving field of medicine called mitochondrial medicine [17, 18, 19, 20]. …”

Section: Introductionmentioning

confidence: 99%