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  1. Is spinal muscular atrophy the result of defects in motor neuron processes?Michael Briese, Behrooz Esmaeili & David B. Sattelle - 2005 - Bioessays 27 (9):946-957.
    The hereditary neurodegenerative disease spinal muscular atrophy (SMA) with childhood onset is one of the most common genetic causes of infant mortality. The disease is characterized by selective loss of spinal cord motor neurons leading to muscle atrophy and is the result of mutations in the survival motor neuron (SMN) gene. The SMN protein has been implicated in diverse nuclear processes including splicing, ribosome formation and gene transcription. Even though the genetic basis of SMA is well understood, it is not (...)
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  • A metabolic enzyme doing double duty as a transcription factor.Anjana Bhardwaj & Miles F. Wilkinson - 2005 - Bioessays 27 (5):467-471.
    Many kinds of multifunctional regulatory proteins have been identified that perform distinct biochemical functions in the nucleus, the cytoplasm, or both. Here we describe the recent discovery by Hall et al. (2004)1 of a new type of multifunctional protein: a metabolic enzyme that doubles as a transcription factor. This enzyme, Arg5,6, functions as a catalytic enzyme in ornithine biosynthesis and also binds and regulates the promoters of nuclear and mitochondrial genes. It may also regulate precursor mRNA metabolism. We discuss how (...)
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