Is spinal muscular atrophy a disease of the motor neurons only: pathogenesis and therapeutic implications?

Spinal muscular atrophy (SMA) is a genetic neurological disease that causes infant mortality; no effective therapies are currently available. SMA is due to homozygous mutations and/or deletions in the survival motor neuron 1 gene and subsequent reduction of the SMN protein, leading to the death of motor neurons. However, there is increasing evidence that in addition to motor neurons, other cell types are contributing to SMA pathology. In this review, we will discuss the involvement of non-motor neuronal cells, located both inside and outside the central nervous system, in disease onset and progression. Even if SMN restoration in motor neurons is needed, it has been shown that optimal phenotypic amelioration in animal models of SMA requires a more widespread SMN correction. It has been demonstrated that non-motor neuronal cells are also involved in disease pathogenesis and could have important therapeutic implications. For these reasons it will be crucial to take this evidence into account for the clinical translation of the novel therapeutic approaches

Optimising the spatial planning of prescribed burns to achieve multiple objectives in a fire-dependent ecosystem

1. There is potential for negative consequences for the ecological integrity of fire-dependent ecosystems as a result of inappropriate fire regimes. This can occur when asset (property) protection is prioritised over conservation objectives in burn programs

Black Hole Thermodynamics and Statistical Mechanics

We have known for more than thirty years that black holes behave as thermodynamic systems, radiating as black bodies with characteristic temperatures and entropies. This behavior is not only interesting in its own right; it could also, through a statistical mechanical description, cast light on some of the deep problems of quantizing gravity. In these lectures, I review what we currently know about black hole thermodynamics and statistical mechanics, suggest a rather speculative "universal" characterization of the underlying states, and describe some key open questions.Comment: 35 pages, Springer macros; for the Proceedings of the 4th Aegean Summer School on Black Hole