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Nonsense-mediated mRNA decay (NMD) is a surveillance pathway that exists in all eukaryotes. Its main function is to reduce errors in gene expression by eliminating mRNA transcripts that contain premature stop codons.If these aberrant mRNA transcripts were translated, the result would be deleterious gain-of-function or dominant-negative activity of the resulting proteins.onsense-mediated decay mutations can also contribute to Marfan syndrome. This disorder is caused by mutations in the fibrillin 1 (FBN1) gene and is resulted from a dominant negative interaction between mutant and wild-type fibrillin-1 gene.
Motor Neurone Disease (MND) is the name given to a group of closely related disorders affecting the motor neurones (nerve cells controlling muscles) in the brain and spinal cord. Degeneration of these motor neurones results in progressive muscle wasting and weakness because the muscles have lost their nerve supply.ecause MND is a progressive disease, the muscular weakness becomes worse with time. The patient may eventually require a wheelchair because of leg and trunk weakness, and become generally immobile. At some stage in the disease the patient will suffer from âbulbarâ symptoms.