Robert Horvitz

Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease that is inherited as an autosomal dominant trait in approximately 10% of cases. Recently we and others identified several single-base mutations in the Cu/Zn superoxide dismutase (SOD1) gene in patients with familial ALS (FALS). Using single-strand conformational polymorphism, we studied the C to G mutation in exon 2 of the SOD1 gene (resulting in a leucine to valine substitution in position 38) in affected and unaffected members of a large Belgian family with FALS. We measured the SOD1 activity in red blood cell lysates in 14 members of this family, including the only surviving clinically affected patient. SOD1 activity of the family members carrying the mutation was less than half that of members without the mutation. In addition, in 11 patients with sporadic ALS and 11 age- and sex-matched controls, red blood cell SOD1 activity was normal. These studies indicate that SOD1 activity is reduced in these FALS patients but not in sporadic ALS patients. Moreover, this SOD1 enzyme abnormality is detectable years before onset of clinical ALS in carriers of this FALS mutation.

The development of a multicellular organism involves the generation of many different cell types from a single-celled fertilized egg. An understanding of the molecular mechanisms that cause cells to become different from one another is a fundamental goal of developmental biology. One organism well suited for the study of this problem at the level of single cells and individual cell divisions is the nematode Caenorhabditis elegans.

Abstract:This is a background paper for an ITU Workshop on "Market Mechanismsfor Spectrum Management" (Geneva, Switzerland, 22-23 January 2007). Itsummarizes the Open Spectrum Foundation's global survey of Wi-Firegulations, with "thumbnail sketches" of the rules in over 170countries. It proposes a new ITU Study Question on how to reconcilethe growing recognition of licence-free radio bands as a "bestpractice" in spectrum management with the long-standing rule (nowfound in S18.1 of the International Radio Regulations) that allnongovernment radio transmitters must be licenced. Finally, itdiscusses the implications of regulators having the ability to createnew authorisation classes in the gap between licenced and unlicenced.That shifts the debate between proponents of (unlicenced) "openspectrum" - and the proponents of (licenced) spectrum as tradableproperty - onto unstable, unfamiliar ground. It could lead to"multi-dimensional bidding" in spectrum auctions - as is foundsometimes in government procurement tenders where bidders can combineprice offers and quality/performance commitments. It may also lead toan understanding of spectrum access rights as a continuum which can belinked to efficiency or politeness, and not just to willingness topay.