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2017 ; 14
(6
): 712-725
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Cajal bodies in neurons
#MMPMID27627892
Lafarga M
; Tapia O
; Romero AM
; Berciano MT
RNA Biol
2017[Jun]; 14
(6
): 712-725
PMID27627892
show ga
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.
|Animals
[MESH]
|Cell Nucleolus/metabolism
[MESH]
|Coiled Bodies/*metabolism
[MESH]
|Disease Susceptibility
[MESH]
|Humans
[MESH]
|Nervous System Diseases/genetics/metabolism/pathology
[MESH]