PMID- 11158314
OWN - NLM
STAT- MEDLINE
DCOM- 20010322
LR  - 20181113
IS  - 0270-7306 (Print)
IS  - 1098-5549 (Electronic)
IS  - 0270-7306 (Linking)
VI  - 21
IP  - 4
DP  - 2001 Feb
TI  - The CELF family of RNA binding proteins is implicated in cell-specific and 
      developmentally regulated alternative splicing.
PG  - 1285-96
AB  - Alternative splicing of cardiac troponin T (cTNT) exon 5 undergoes a 
      developmentally regulated switch such that exon inclusion predominates in 
      embryonic, but not adult, striated muscle. We previously described four 
      muscle-specific splicing enhancers (MSEs) within introns flanking exon 5 in 
      chicken cTNT that are both necessary and sufficient for exon inclusion in 
      embryonic muscle. We also demonstrated that CUG-binding protein (CUG-BP) binds a 
      conserved CUG motif within a human cTNT MSE and positively regulates 
      MSE-dependent exon inclusion. Here we report that CUG-BP is one of a novel family 
      of developmentally regulated RNA binding proteins that includes embryonically 
      lethal abnormal vision-type RNA binding protein 3 (ETR-3). This family, which we 
      call CELF proteins for CUG-BP- and ETR-3-like factors, specifically bound 
      MSE-containing RNAs in vitro and activated MSE-dependent exon inclusion of cTNT 
      minigenes in vivo. The expression of two CELF proteins is highly restricted to 
      brain. CUG-BP, ETR-3, and CELF4 are more broadly expressed, and expression is 
      developmentally regulated in striated muscle and brain. Changes in the level of 
      expression and isoforms of ETR-3 in two different developmental systems 
      correlated with regulated changes in cTNT splicing. A switch from cTNT exon 
      skipping to inclusion tightly correlated with induction of ETR-3 protein 
      expression during differentiation of C2C12 myoblasts. During heart development, 
      the switch in cTNT splicing correlated with a transition in ETR-3 protein 
      isoforms. We propose that ETR-3 is a major regulator of cTNT alternative splicing 
      and that the CELF family plays an important regulatory role in cell-specific 
      alternative splicing during normal development and disease.
FAU - Ladd, A N
AU  - Ladd AN
AD  - Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA.
FAU - Charlet, N
AU  - Charlet N
FAU - Cooper, T A
AU  - Cooper TA
LA  - eng
SI  - GENBANK/AF329264
SI  - GENBANK/AF329265
SI  - GENBANK/AF329266
GR  - R01 AR045653/AR/NIAMS NIH HHS/United States
GR  - AR45653/AR/NIAMS NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Mol Cell Biol
JT  - Molecular and cellular biology
JID - 8109087
RN  - 0 (CELF Proteins)
RN  - 0 (CELF1 Protein)
RN  - 0 (CELF1 protein, human)
RN  - 0 (CELF2 protein, human)
RN  - 0 (CELF3 protein, human)
RN  - 0 (CELF4 protein, human)
RN  - 0 (CELF5 protein, human)
RN  - 0 (DNA Primers)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (RNA-Binding Proteins)
RN  - 0 (Ribonucleoproteins)
RN  - 0 (Troponin T)
SB  - IM
MH  - Adult
MH  - *Alternative Splicing
MH  - Amino Acid Sequence
MH  - Animals
MH  - Base Sequence
MH  - Brain/growth & development/metabolism
MH  - CELF Proteins
MH  - CELF1 Protein
MH  - Cell Line
MH  - Chickens
MH  - DNA Primers/genetics
MH  - Enhancer Elements, Genetic
MH  - Exons
MH  - Female
MH  - Gene Expression
MH  - Humans
MH  - Male
MH  - Molecular Sequence Data
MH  - Muscle Development
MH  - Muscle, Skeletal/growth & development/metabolism
MH  - Nerve Tissue Proteins
MH  - RNA-Binding Proteins/*genetics/*metabolism
MH  - Ribonucleoproteins/genetics
MH  - Sequence Homology, Amino Acid
MH  - Tissue Distribution
MH  - Troponin T/genetics
PMC - PMC99581
EDAT- 2001/02/07 11:00
MHDA- 2001/03/27 10:01
PMCR- 2001/02/01
CRDT- 2001/02/07 11:00
PHST- 2001/02/07 11:00 [pubmed]
PHST- 2001/03/27 10:01 [medline]
PHST- 2001/02/07 11:00 [entrez]
PHST- 2001/02/01 00:00 [pmc-release]
AID - 1566 [pii]
AID - 10.1128/MCB.21.4.1285-1296.2001 [doi]
PST - ppublish
SO  - Mol Cell Biol. 2001 Feb;21(4):1285-96. doi: 10.1128/MCB.21.4.1285-1296.2001.