Abstract: Cajal is commonly regarded as the father of modern neuroscience in recognition of his fundamental work
on the structure of the nervous system. But Cajal also made seminal contributions to the knowledge of
nuclear structure in the early 1900s, including the discovery of the ?accessory body? later renamed ?Cajal
body? (CB). This important nuclear structure has emerged as a center for the assembly of
ribonucleoproteins (RNPs) required for splicing, ribosome biogenesis and telomere maintenance. The
modern era of CB research started in the 1990s with the discovery of coilin, now known as a scaffold
protein of CBs, and specific probes for small nuclear RNAs (snRNAs). In this review, we summarize what we
have learned in the recent decades concerning CBs in post-mitotic neurons, thereby ruling out dynamic
changes in CB functions during the cell cycle. We show that CBs are particularly prominent in neurons,
where they frequently associate with the nucleolus. Neuronal CBs are transcription-dependent nuclear
organelles. Indeed, their number dynamically accommodates to support the high neuronal demand for
splicing and ribosome biogenesis required for sustaining metabolic and bioelectrical activity. Mature
neurons have canonical CBs enriched in coilin, survival motor neuron protein and snRNPs. Disruption and
loss of neuronal CBs associate with severe neuronal dysfunctions in several neurological disorders such as
motor neuron diseases. In particular, CB depletion in motor neurons seems to reflect a perturbation of
transcription and splicing in spinal muscular atrophy, the most common genetic cause of infant mortality.
Otras publicaciones de la misma revista o congreso con autores/as de la Universidad de Cantabria
Fuente: RNA Biol. 2017 Jun 3;14(6):712-725
Editorial: Taylor & Francis
Fecha de publicación: 01/06/2017
Nº de páginas: 14
Tipo de publicación: Artículo de Revista
DOI: 10.1080/15476286.2016.1231360
ISSN: 1547-6286,1555-8584