News & Comment
TRENDS in Molecular Medicine Vol.7 No.1 January 2001
11
In Brief
Eyeing-up stem cell transplantation
who both have breast cancer. Collections will be nationally available through regional banking facilities. Details are available at the MRC website (http://www.mrc.ac.uk). (AR)
Idiopathic Down’s syndrome cluster in Ireland
The recent identification of neural stem cells has encouraged scientists that these undifferentiated progenitor cells might hold the key to treatment of many neurodegenerative disorders. This possibility took a step forward when researchers led by Don Sakaguchi (Iowa State University, IA) and Michael Young Harvard Medical School, MA, USA) successfully transplanted and integrated stem cells into the eyes of postnatal Brazillian opossums. Spheres of green-fluorescent protein (GFP) expressing rat hippocampal-derived or embryonic mouse stem cells were injected into the eyes of opossum pups. Immunoreactivity for several neural and retinal markers was observed, indicating that these transplanted cells had integrated into the eye and differentiated into retinal neurons. Integration and survival of these cells raises the prospect of treatment for human retinopathies such as glaucoma and macular degeneration. (AR)
MRC funding for DNA collections In a new initiative to identify genes that cause common diseases, the UK Medical Research Council (MRC) has announced that it is to fund 14 new DNA collections at a cost of £8.3m. Conditions such as heart disease, diabetes and mental health disorders, which have strong genetic components, will be studied using large collections of DNA from patients who are being treated for specific diseases, and DNA from unaffected individuals. For example, the genetic predisposition to breast cancer, which kills 12 000 women in the UK per year, is being investigated using a DNA panel taken from 500 pairs of sisters
Molecular analysis has contributed to the conclusion that a 30-fold increase in the number of Down’s syndrome children born to mothers who attended the same girls’ school in Dundalk, Ireland between 1956 and 1957 is almost certainly the result of pure chance. Out of 387 births to former pupils, six had Down’s syndrome. Previously, blame has been placed on either radioactive fallout from the Windscale nuclear power station in England (now called Sellafield), or a ‘flu epidemic in 1957. Norman Nevin (Belfast City Hospital, Belfast, UK) and colleagues performed microsatellite analysis on DNA taken from parents and affected children, and demonstrated that in one individual, nondisjunction was the result of a postfertilization mitotic error, thus excluding this case from a common environmental cause. Furthermore, it appeared that not all of the mothers were even at the school at the same time, and that some of them had moved away from the area when the fallout or ‘flu epidemic events occurred, leading to the conclusion that chance is the most likely explanation for this anomaly. (AR)
Lasers aimed at mad cows Protein misfolding can lead to neurodegenerative diseases such as Parkinson’s, Alzheimer’s and Creutzfeldt–Jakob disease (CJD). A new technique designed by Laurence Barron (Glasgow University, UK) has revealed a conformational change in a model protein that results in fibrillar aggregation, similar to that seen in diseased nerve tissue. The technique – Raman Optical Activity spectroscopy (ROA) – bypasses the difficulties of protein crystallization, and involves shining lasers at proteins in solution and measuring chirality vibrational spectra. The identification of the same structural change – polyproline II helix – in proteins implicated in neurodegenerative diseases might reveal how altered molecular architecture is involved in these fatal conditions. (AR)
Scaffolding in pigs offers cardiovascular gene therapy A new technique for delivering DNA to damaged arteries may prove useful for future gene therapy protocols aimed at treating injured coronary arteries and heart disease. Robert Levy’s team at the Children’s Hospital of Philadelphia used tiny metal scaffolds called ‘stents’ to deliver genes to cells in the artery wall in pigs. Stents are currently used extensively in angioplasty procedures to support arterial walls following inflation of blocked coronary arteries. Stent-based gene delivery could be important to control blood vessel damage and obstructive cell growth observed in over 30% of angioplasty patients. The researchers immobilized DNA within a biodegradable film coating the metal stents allowing localized, controlled release and delivery of genes. This approach has advantages over current systemic delivery systems used for gene therapy. Further work is required to identify genes that would be most beneficial for arterial stent-based delivery. (JW)
Cannibalism reveals vCJDsusceptibility All the 88 victims of vCJD in the UK are believed to have been homozygous for a polymorphism in the gene encoding the prion protein PrPC. 37% of the UK population have a methionine, rather than a valine, residue at codon 129 in both alleles, and it has been thought that heterozygosity at this locus may be protective. However, recent findings have shown that 11 elderly victims of a different form of CJD called Kuru were heterozygous at codon 129. UK government adviser John Collinge (St. Mary’s Hospital, London, UK) presented data at the Millennium Festival of Medicine (London, UK) that implied that heterozygosity does not confer immunity, but augments the incubation period of Kuru. The ceremonial eating of dead relatives’ brains is thought to be responsible for over 2500 Kuru deaths in the Fore tribespeople of Papua New Guinea in the 20th century. This practice has been outlawed since 1957, but the prolonged incubation period has allowed the identification of elderly heterozygous victims. (AR)
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