http://www.cnr.it/ontology/cnr/individuo/prodotto/ID4171
Chaperoning ribonucleoprotein biogenesis in health and disease (Articolo in rivista)
- Type
- Label
- Chaperoning ribonucleoprotein biogenesis in health and disease (Articolo in rivista) (literal)
- Anno
- 2007-01-01T00:00:00+01:00 (literal)
- Alternative label
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Pagina inizio
- Pagina fine
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
- Rivista
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#descrizioneSinteticaDelProdotto
- Pubblicazione su rivista internazionale (literal)
- Note
- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Titolo
- Chaperoning ribonucleoprotein biogenesis in health and disease (literal)
- Abstract
- The survival motor neuron (SMN) protein is part of a macromolecular
complex that functions in the biogenesis of small
nuclear ribonucleoproteins (snRNPs)the essential components
of the pre-messenger RNA splicing machineryas well as probably
other RNPs. Reduced levels of SMN expression cause the
inherited motor neuron disease spinal muscular atrophy (SMA).
Knowledge of the composition, interactions and functions of the
SMN complex has advanced greatly in recent years. The emerging
picture is that the SMN complex acts as a macromolecular
chaperone of RNPs to increase the efficiency and fidelity of RNA
protein interactions in vivo, and to provide an opportunity for
these interactions to be regulated. In addition, it seems that RNA
metabolism deficiencies underlie SMA. Here, a dual dysfunction
hypothesis is presented in which two mechanistically and temporally
distinct defectsthat are dependent on the extent of SMN
reduction in SMAaffect the homeostasis of specific messenger
RNAs encoding proteins essential for motor neuron development
and function. (literal)
- Prodotto di
- Autore CNR
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