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In most peroxisomal disorders the nervous system is severely affected which explains the clinical and community burden they represent. This is the first book to focus not only on the mutations causing these inherited illnesses, but also on mechanisms that regulate, suppress or enhance expression of genes and their products (enzymes). Indeed since the success and completion of the Human Genome Project all genes (coding DNA sequences) are known. However, of many, their function, and the role of the gene product has not been determined. An example is X-linked adrenoleukodystrophy, the most frequent peroxisomal disorder. Children are born healthy, but in more than 1 out of 3, demyelination of the brain starts unpredictably and they die in a vegetative state. The gene mutated in most families has been known for 10 years; but the true role of the encoded protein, ALDp, is still speculative; and within the same family, very severe and asymptomatic clinical histories co-exist, unexplained by the mutation.
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The coming of the railways signalled the transformation of European society, allowing the quick and cheap mass transportation of people and goods on a previously unimaginable scale. By the early decades of the twentieth century, however, the domination of rail transport was threatened by increased motorised road transport which would quickly surpass and eclipse the trains, only itself to be challenged in the twenty-first century by a renewal of interest in railways. Yet, as the studies in this volume make clear, to view the relationship between road and rail as a simple competition between two rival forms of transportation, is a mistake. Rail transport did not vanish in the twentieth century...