The feasibility of making short-term migration estimates

Short-term migration, that is stays of less than 12 months, has received particular attention recently from both national and local level users of population statistics. This interest is part of the wider interest shown in increasing levels of long-term migration statistics in recent years. This article is an initial assessment by ONS of the potential to produce short-term migration estimates for England and Wales, and the challenges faced in doing so. Central to these challenges is the question of how short-term migration should be defined. In addition, illustrative estimates of short-term migration based on the International Passenger Survey (IPS) are provided. These illustrative estimates, along with associated standard errors, are provided at both national and regional levels using a number of definitional bases.     

Using administrative data sources in the estimation of emigration

It is important that migration is measured accurately, for example to inform population estimates and projections. However, current sources of information present challenges in producing robust estimates of emigration from Great Britain. This article reports on work carried out by the Office for National Statistics to investigate the potential for using administrative data sources to contribute to the measurement of emigration.     

Phytanic acid impairs mitochondrial respiration through protonophoric action

Refsum disease is a rare, inherited neurodegenerative disorder characterized by accumulation of the dietary branched-chain fatty acid phytanic acid in plasma and tissues caused by a defect in the alphaoxidation pathway. The accumulation of phytanic acid is believed to be the main pathophysiological cause of the disease. However, the exact mechanism(s) by which phytanic acid exerts its toxicity have not been resolved. In this study, the effect of phytanic acid on mitochondrial respiration was investigated. The results show that in digitonin-permeabilized fibroblasts, phytanic acid decreases ATP synthesis, whereas substrate oxidation per se is not affected. Importantly, studies in intact fibroblasts revealed that phytanic acid decreases both the mitochondrial membrane potential and NAD(P)H autofluorescence. Taken together, the results described here show that unesterified phytanic acid exerts its toxic effect mainly through its protonophoric action, at least in human skin fibroblasts. Received 4 August 2007; received after revision 26 September 2007; accepted 10 October 2007 J. C. Komen, F. Distelmaier: These authors contributed equally to this work.     

Mutations in TTBK2, encoding a kinase implicated in tau phosphorylation, segregate with spinocerebellar ataxia type 11

The microtubule-associated protein tau (encoded by MAPT) and several tau kinases have been implicated in neurodegeneration, but only MAPT has a proven role in disease. We identified mutations in the gene encoding tau tubulin kinase 2 (TTBK2) as the cause of spinocerebellar ataxia type 11. Affected brain tissue showed substantial cerebellar degeneration and tau deposition. These data suggest that TTBK2 is important in the tau cascade and in spinocerebellar degeneration.