[HTML][HTML] Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarin
KW Jones, K Gorzynski, CM Hales, U Fischer… - Journal of Biological …, 2001 - ASBMB
Disruption of the survival motor neuron (SMN) gene leads to selective loss of spinal motor
neurons, resulting in the fatal human neurodegenerative disorder spinal muscular atrophy
(SMA). SMN has been shown to function in spliceosomal small nuclear ribonucleoprotein
(snRNP) biogenesis and pre-mRNA splicing. We have demonstrated that SMN also interacts
with fibrillarin, a highly conserved nucleolar protein that is associated with all Box C/D small
nucleolar RNAs and functions in processing and modification of rRNA. Fibrillarin and SMN …
neurons, resulting in the fatal human neurodegenerative disorder spinal muscular atrophy
(SMA). SMN has been shown to function in spliceosomal small nuclear ribonucleoprotein
(snRNP) biogenesis and pre-mRNA splicing. We have demonstrated that SMN also interacts
with fibrillarin, a highly conserved nucleolar protein that is associated with all Box C/D small
nucleolar RNAs and functions in processing and modification of rRNA. Fibrillarin and SMN …