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Physiol. Genomics (October 15, 2002). doi:10.1152/physiolgenomics.00100.2002
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Articles in PresS, published online ahead of print October 15, 2002
Physiol Genomics, 10.1152/physiolgenomics.00100.2002
Submitted on August 6, 2002
Accepted on September 30, 2002

Vitamin E deficiency and metabolic deficits in neuronal ceroid lipofuscinosis described by bioinformatics

Julian L Griffin1*, David Muller2, Reshma Woograsingh1, Victoria Jowatt3, Andrew Hindmarsh3, Jeremy K Nicholson1, and Joanne E Martin3

1 Biomedical Sciences, Imperial College of Science, Technology and Medicine, London, United Kingdom
2 Institute of Child Health, London, United Kingdom
3 Histopathology, The Royal London Hospital, London, United Kingdom

* To whom correspondence should be addressed. E-mail: j.griffin{at}ic.ac.uk.

The mnd mouse, a model of neuronal ceroid lipofusinosis (NCL), has a profound vitamin E deficiency in sera and brain, associated with cerebral deterioration characteristic of NCL. In this study, the vitamin E deficiency is corrected using dietary supplementation. However, the histopathological features associated with NCL remained. Using a bioinformatics approach based on high resolution solid and solution state 1H NMR spectroscopy and principal component analysis (PCA), the deficits associated with NCL are defined in terms of a metabolic phenotype. While vitamin E supplementation reversed some of the metabolic abnormalities, in particular the concentration of phenylalanine in extracts of cerebral tissue, PCA demonstrated that metabolic deficits associated with NCL were greater than any effects produced from vitamin E supplementation. These deficits included increased glutamate and NAA and decreased creatine and glutamine concentrations in aqueous extracts of the cortex, as well as profound accumulation of lipid in intact cerebral tissue. This is discussed in terms of faulty production of mitochondrial associated membranes, thought to be central to the deficits in mnd mice.




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