The SR protein family of splicing factors: master regulators of gene expression

被引:867
作者
Long, Jennifer C. [1 ]
Caceres, Javier F. [1 ]
机构
[1] Western Gen Hosp, Inst Genet & Mol Med, MRC, Human Genet Unit, Edinburgh EH4 2XU, Midlothian, Scotland
基金
英国医学研究理事会;
关键词
alternative splicing; pre-mRNA splicing; RS domain; SR proteins; SR-related proteins; translation regulation; PRE-MESSENGER-RNA; SPINAL MUSCULAR-ATROPHY; U4/U6-CENTER-DOT-U5; TRI-SNRNP; SERINE/ARGININE-RICH PROTEINS; GENOME-WIDE SURVEY; SITE SELECTION; IN-VIVO; RS DOMAIN; PREMESSENGER RNA; FACTOR ASF/SF2;
D O I
10.1042/BJ20081501
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The SR protein family comprises a number of phylogenetically conserved and structurally related proteins with a characteristic domain rich in arginine and serine residues, known as the RS domain. They play significant roles ill constitutive pre-mRNA splicing and are also important regulators of alternative splicing. In addition they participate in post-splicing activities, such as mRNA nuclear export, nonsense-mediated ill RNA decay and mRNA translation. These wide-ranging roles of SR proteins highlight their importance as pivotal regulators of mRNA metabolism, and if these functions are disrupted, developmental defects or disease may result. Furthermore, animal models have shown a highly specific, non-redundant role for individual SR proteins in the regulation of developmental processes. Here, we will review the Current literature to demonstrate how SR proteins are emerging as one of the master regulators of gene expression.
引用
收藏
页码:15 / 27
页数:13
相关论文
共 215 条
[21]   Broad specificity of SR (serine/arginine) proteins in the regulation of alternative splicing of pre-messenger RNA [J].
Bourgeois, CF ;
Lejeune, F ;
Stévenin, J .
PROGRESS IN NUCLEIC ACID RESEARCH AND MOLECULAR BIOLOGY, VOL 78, 2004, 78 :37-88
[22]   RNA folding affects the recruitment of SR proteins by mouse and human polypurinic enhancer elements in the fibronectin EDA dxon [J].
Buratti, E ;
Muro, AF ;
Giombi, M ;
Gherbassi, D ;
Iaconcig, A ;
Baralle, FE .
MOLECULAR AND CELLULAR BIOLOGY, 2004, 24 (03) :1387-1400
[23]   SR protein-mediated inhibition of CFTR exon 9 inclusion: molecular characterization of the intronic splicing silencer [J].
Buratti, Ernanuele ;
Stuani, Cristiana ;
De Prato, Greta ;
Baralle, Francisco E. .
NUCLEIC ACIDS RESEARCH, 2007, 35 (13) :4359-4368
[24]   REGULATION OF ALTERNATIVE SPLICING IN-VIVO BY OVEREXPRESSION OF ANTAGONISTIC SPLICING FACTORS [J].
CACERES, JF ;
STAMM, S ;
HELFMAN, DM ;
KRAINER, AR .
SCIENCE, 1994, 265 (5179) :1706-1709
[25]   Role of the modular domains of SR proteins in subnuclear localization and alternative splicing specificity [J].
Caceres, JF ;
Misteli, T ;
Screaton, GR ;
Spector, DL ;
Krainer, AR .
JOURNAL OF CELL BIOLOGY, 1997, 138 (02) :225-238
[26]   A specific subset of SR proteins shuttles continuously between the nucleus and the cytoplasm [J].
Cáceres, JF ;
Screaton, GR ;
Krainer, AR .
GENES & DEVELOPMENT, 1998, 12 (01) :55-66
[27]  
Cao WH, 1997, RNA, V3, P1456
[28]   Listening to silence and understanding nonsense: Exonic mutations that affect splicing [J].
Cartegni, L ;
Chew, SL ;
Krainer, AR .
NATURE REVIEWS GENETICS, 2002, 3 (04) :285-298
[29]   Correction of disease-associated exon skipping by synthetic exon-specific activators [J].
Cartegni, L ;
Krainer, AR .
NATURE STRUCTURAL BIOLOGY, 2003, 10 (02) :120-125
[30]   Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1 [J].
Cartegni, L ;
Krainer, AR .
NATURE GENETICS, 2002, 30 (04) :377-384