Functions of the fragile X protein

被引:10
作者
Oostra, BA [1 ]
机构
[1] Erasmus Univ, CBG Dept Clin Genet, NL-3000 DR Rotterdam, Netherlands
关键词
D O I
10.1016/S1471-4914(02)02271-2
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
Fragile X syndrome is caused by the absence of the FMR1 protein (FMRP). FMRP is an RNA-binding protein that can bind 4% of mRNA species. Several of those mRNAs have now been identified. FMRP binds to its own mRNA to a sequence containing a G quartet structure -a structure also found in some of the other mRNAs. This interesting new finding, which has used clues from a Drosophila fragile X model, are discussed here.
引用
收藏
页码:102 / 103
页数:2
相关论文
共 15 条
[1]   FMR1 PROTEIN - CONSERVED RNP FAMILY DOMAINS AND SELECTIVE RNA-BINDING [J].
ASHLEY, CT ;
WILKINSON, KD ;
REINES, D ;
WARREN, ST .
SCIENCE, 1993, 262 (5133) :563-566
[2]   Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome [J].
Brown, V ;
Jin, P ;
Ceman, S ;
Darnell, JC ;
O'Donnell, WT ;
Tenenbaum, SA ;
Jin, XK ;
Feng, Y ;
Wilkinson, KD ;
Keene, JD ;
Darnell, RB ;
Warren, ST .
CELL, 2001, 107 (04) :477-487
[3]   Abnormal dendritic spines in fragile X knockout mice: Maturation and pruning deficits [J].
Comery, TA ;
Harris, JB ;
Willems, PJ ;
Oostra, BA ;
Irwin, SA ;
Weiler, IJ ;
Greenough, WT .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1997, 94 (10) :5401-5404
[4]   Fragile X mental retardation protein targets G quartet mRNAs important for neuronal function [J].
Darnell, JC ;
Jensen, KB ;
Jin, P ;
Brown, V ;
Warren, ST ;
Darnell, RB .
CELL, 2001, 107 (04) :489-499
[5]   A POINT MUTATION IN THE FMR-1 GENE ASSOCIATED WITH FRAGILE-X MENTAL-RETARDATION [J].
DEBOULLE, K ;
VERKERK, AJMH ;
REYNIERS, E ;
VITS, L ;
HENDRICKX, J ;
VANROY, B ;
VANDENBOS, F ;
DEGRAAFF, E ;
OOSTRA, BA ;
WILLEMS, PJ .
NATURE GENETICS, 1993, 3 (01) :31-35
[6]   The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals [J].
Eberhart, DE ;
Malter, HE ;
Feng, Y ;
Warren, ST .
HUMAN MOLECULAR GENETICS, 1996, 5 (08) :1083-1091
[7]   The fragile X mental retardation protein is associated with ribosomes [J].
Khandjian, EW ;
Corbin, F ;
Woerly, S ;
Rousseau, F .
NATURE GENETICS, 1996, 12 (01) :91-93
[8]   Comparative genomic sequence analysis of the FXR gene family:: FMR1, FXR1, and FXR2 [J].
Kirkpatrick, LL ;
McIlwain, KA ;
Nelson, DL .
GENOMICS, 2001, 78 (03) :169-177
[9]   Fragile X syndrome at the turn of the century [J].
Kooy, RF ;
Willemsen, R ;
Oostra, BA .
MOLECULAR MEDICINE TODAY, 2000, 6 (05) :193-198
[10]   Evidence that fragile X mental retardation protein is a negative regulator of translation [J].
Laggerbauer, B ;
Ostareck, D ;
Keidel, EM ;
Ostareck-Lederer, A ;
Fischer, U .
HUMAN MOLECULAR GENETICS, 2001, 10 (04) :329-338