TY - JOUR
T1 - SMN interacts with a novel family of hnRNP and spliceosomal proteins
AU - Mourelatos, Zissimos
AU - Abel, Linda
AU - Yong, Jeongsik
AU - Kataoka, Naoyuki
AU - Dreyfuss, Gideon
PY - 2001/10/1
Y1 - 2001/10/1
N2 - Spinal muscular atrophy (SMA) is a common neuro-degenerative disease caused by deletion or loss-of-function mutations of the survival of motor neurons (SMN) protein. SMN is in a complex with several proteins, including Gemin2, Gemin3 and Gemin4, and it plays important roles in small nuclear ribonucleo-protein (snRNP) biogenesis and in pre-mRNA splicing. Here, we characterize three new hnRNP proteins, collectively referred to as hnRNP Qs, which are derived from alternative splicing of a single gene. The hnRNP Q proteins interact with SMN, and the most common SMN mutant found in SMA patients is defective in its interactions with them. We further demonstrate that hnRNP Qs are required for efficient pre-mRNA splicing in vitro. The hnRNP Q proteins may provide a molecular link between the SMN complex and splicing.
AB - Spinal muscular atrophy (SMA) is a common neuro-degenerative disease caused by deletion or loss-of-function mutations of the survival of motor neurons (SMN) protein. SMN is in a complex with several proteins, including Gemin2, Gemin3 and Gemin4, and it plays important roles in small nuclear ribonucleo-protein (snRNP) biogenesis and in pre-mRNA splicing. Here, we characterize three new hnRNP proteins, collectively referred to as hnRNP Qs, which are derived from alternative splicing of a single gene. The hnRNP Q proteins interact with SMN, and the most common SMN mutant found in SMA patients is defective in its interactions with them. We further demonstrate that hnRNP Qs are required for efficient pre-mRNA splicing in vitro. The hnRNP Q proteins may provide a molecular link between the SMN complex and splicing.
KW - HnRNP proteins
KW - Pre-mRNA splicing
KW - Spinal muscular atrophy
KW - Survival of motor neurons
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U2 - 10.1093/emboj/20.19.5443
DO - 10.1093/emboj/20.19.5443
M3 - Article
C2 - 11574476
AN - SCOPUS:0035476679
SN - 0261-4189
VL - 20
SP - 5443
EP - 5452
JO - EMBO Journal
JF - EMBO Journal
IS - 19
ER -