Cultured muscle cells display defects of mitochondrial myopathy ameliorated by anti-oxidants

The mitochondrial DNA A3243G mutation causes neuromuscular disease. To investigate the muscle-specific pathophysiology of mitochondrial disease, rhabdomyosarcoma transmitochondrial hybrid cells (cybrids) were generated that retain the capacity to differentiate to myotubes. In some cases, striated muscle-like fibres were formed after innervation with rat embryonic spinal cord. Myotubes carrying A3243G mtDNA produced more reactive oxygen species than controls, and had altered glutathione homeostasis. Moreover, A3243G mutant myotubes showed evidence of abnormal mitochondrial distribution, which was associated with down-regulation of three genes involved in mitochondrial morphology, Mfn1, Mfn2 and DRP1. Electron microscopy revealed mitochondria with ultrastructural abnormalities and paracrystalline inclusions. All these features were ameliorated by anti-oxidant treatment, with the exception of the paracrystalline inclusions. These data suggest that rhabdomyosarcoma cybrids are a valid cellular model for studying muscle-specific features of mitochondrial disease and that excess reactive oxygen species production is a significant contributor to mitochondrial dysfunction, which is amenable to anti-oxidant therapy.

A comparison of maternal calcium and magnesium levels in pre-eclamptic and normotensive pregnancies: an observational case–control study

Objective : Supplementing pregnant women at high risk of developing pre-eclampsia with calcium may reduce the incidence of the disease. This study examines differences in serum and hair concentrations of calcium and magnesium between women with pre-eclamptic and normotensive pregnancies.Design : Observational case–control study.Setting : Two teaching hospitals in Cape Town, South Africa.PopulationWomen with pre-eclamptic (N = 96) or normotensive (N = 96) pregnancies, who delivered a single, live infant.MethodsDemographic and current pregnancy details were retrieved from clinical notes. Each participant completed a dietary questionnaire. Venous blood samples were taken from each participant to assess serum calcium and magnesium concentrations. Hair samples were obtained from all participants and calcium and magnesium levels were measured by inductively coupled plasma optical emission spectrometry (ICPOES).Main outcome measureHair and serum calcium and magnesium concentrations were compared between women with pre-eclamptic and normotensive pregnancies.ResultsDiet and socio-economic status in the two groups were similar. There was no significant difference in the hair calcium level between women with pre-eclamptic [1241 parts per million (ppm); range, 331–4654 ppm] and normotensive (1146 ppm; range, 480–4136 ppm) pregnancies (P = 0.5). Hair calcium levels in both groups were not affected by HIV infection.ConclusionWoman with pre-eclampsia showed no difference in chronic calcium status relative to normotensive women. This finding does not support the current belief that the mechanism by which calcium supplementation reduces the risk of developing pre-eclampsia is by correcting a nutritional deficiency.

When William Wallace was a unionist

  James Russell Thompson was a successful businessman from Airdrie in Scotland. He arrived in the Victorian gold-mining town of Ballarat in 1853, having previously struck gold on the Ovens goldfields. Deafness caused by his earlier career in mining prevented Thompson from becoming involved in public life in Ballarat but, dying a wealthy man in May 1886, he was able to leave significant bequests to relatives and requested that his remaining estate be put towards the purchase of statues for Ballarat's sprawling botanic gardens. A fellow Scot, Thomas Stoddart, was executor of Thompson's estate, and was able to procure for the gardens numerous monuments and statues made of Italian Carrara marble. The most notable of Stoddart's procurements was the statue of the Scottish hero William Wallace. The Ballarat Star noted that "the statue of Wallace was decided on as a compliment to Mr Thompson's love for the country he came from-an effigy of the greatest character ... in Scottish history or legend". The statue of Wallace in Ballarat's botanic gardens was unveiled and bequeathed to the city on May 24, 1889. It is one of very few outside Scotland.