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Books > Science & Mathematics > Biology, life sciences > Life sciences: general issues > Neurosciences
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RNA Metabolism in Neurodegenerative Diseases (Hardcover, 1st ed. 2018)
Loot Price: R2,915
Discovery Miles 29 150
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RNA Metabolism in Neurodegenerative Diseases (Hardcover, 1st ed. 2018)
Series: Advances in Neurobiology, 20
Expected to ship within 10 - 15 working days
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It has become evident over the last years that abnormalities in RNA
processing play a fundamental part in the pathogenesis of
neurodegenerative diseases. Cellular viability depends on proper
regulation of RNA metabolism and subsequent protein synthesis,
which requires the interplay of many processes including
transcription, pre---mRNA splicing, mRNA editing as well as mRNA
stability, transport and translation. Dysfunction in any of these
processes, often caused by mutations in the coding and non---
coding RNAs, can be very destructive to the cellular environment
and consequently impair neural viability. The result of this RNA
toxicity can lead to a toxic gain of function or a loss of
function, depending on the nature of the mutation. For example, in
repeat expansion disorders, such as the newly discovered
hexanucleotide repeat expansion in theC9orf72 gene found in
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia
(FTD), a toxic gain of function leads to the formation of RNA foci
and the sequestration of RNA binding proteins (RBPs). This in
return leads to a loss of function of those RBPs, which is
hypothesized to play a significant part in the disease progression
of ALS and FTD. Other toxicities arising from repeat expansions are
the formation of RNA foci, bi---directional transcription and
production of repeat associated non---ATG (RAN) translation
products. This book will touch upon most of these disease
mechanisms triggered by aberrant RNA metabolism and will therefore
provide a broad perspective of the role of RNA processing and its
dysfunction in a variety of neurodegenerative disorders, including
ALS, FTD, Alzheimer's disease, Huntington's disease, spinal
muscular atrophy, myotonic dystrophy and ataxias. The proposed
authors are leading scientists in the field and are expected to not
only discuss their own work, but to be inclusive of historic as
well as late breaking discoveries. The compiled chapters will
therefore provide a unique collection of novel studies and
hypotheses aimed to describe the consequences of altered RNA
processing events and its newest molecular players and pathways.
General
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