Magnetic resonance spectroscopy reveals mitochondrial dysfunction in amyotrophic lateral sclerosis.

Mitochondrial dysfunction is postulated to be central to amyotrophic lateral sclerosis (ALS) pathophysiology. Evidence comes primarily from disease models and conclusive data to support bioenergetic dysfunction in vivo in patients is currently lacking. This study is the first to assess mitochondrial dysfunction in brain and muscle in individuals living with ALS using 31P-magnetic resonance spectroscopy (MRS), the modality of choice to assess energy metabolism in vivo.

Light affects mitochondria to cause apoptosis to cultured cells: possible relevance to ganglion cell death in certain optic neuropathies.

Retinal ganglion cell axons within the globe are laden with mitochondria that are unprotected from light (400-760 nm) impinging onto the retina. Light can be absorbed by mitochondrial enzymes such as cytochrome and flavin oxidases causing the generation of reactive oxygen species, and we have suggested this may pose a risk to ganglion cell survival if their energy state is compromised, as may be so in glaucoma or in Leber's Hereditary Optic Neuropathy. Here, we demonstrate that light (400-760 nm) provokes apoptosis in cultured retinal ganglion-5 cells, and that this effect is enhanced in low serum, and attenuated by various antioxidants.

Mitochondrial dysfunction in Parkinson's disease--revisited.

In recent years much of the research conducted in the field of Parkinson's disease (PD) has been aimed at trying to elucidate the molecular mechanisms involved in the development of the rare familial forms of the disease, with the hope that an understanding of these mechanisms will shed some light into the pathogenesis of the more prevalent idiopathic form of the disease. These studies have implicated mitochondrial dysfunction in the pathogenesis of familial PD, either as a primary event, with disease causing mutations identified in genes encoding mitochondrial proteins, or as a secondary event. Thus, the role played by mitochondrial respiratory chain function in the pathogeneis of idiopathic PD, an area of research that was a main focus some 15-20 years ago, has come back to the forefront of the field.