Evidence that fragile X mental retardation protein is a negative regulator of translation

Human Molecular Genetics

Volumn 10, Issue 4, 2001, Pages 329-338

  Laggerbauer, Bernhard ^a   Ostareck, Dirk ^b   Keidel, Eva Maria ^a   Ostareck Lederer, Antje ^b   Fischer, Utz ^a  

^a MAX PLANCK INSTITUTE OF BIOCHEMISTRY   (Germany)

^b EUROPEAN MOLECULAR BIOLOGY LABORATORY   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ASPARAGINE; FRAGILE X MENTAL RETARDATION PROTEIN; ISOLEUCINE; MESSENGER RNA; RNA BINDING PROTEIN; UNCLASSIFIED DRUG;

AMINO ACID SUBSTITUTION; ANIMAL CELL; ARTICLE; AUTOSOME; BINDING AFFINITY; BINDING SITE; CELL LYSATE; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; FRAGILE X SYNDROME; GENE EXPRESSION REGULATION; GENE MUTATION; GENE SILENCING; IN VIVO STUDY; MENTAL DEFICIENCY; MICROINJECTION; NONHUMAN; NUCLEOTIDE SEQUENCE; OLIGOMERIZATION; OOCYTE; PATHOPHYSIOLOGY; POINT MUTATION; POLYSOME; PRIORITY JOURNAL; PROTEIN PROCESSING; PROTEIN PROTEIN INTERACTION; RABBIT; RETICULOCYTE; RIBOSOME SUBUNIT; RNA TRANSLATION; SEQUENCE HOMOLOGY; TRANSLATION REGULATION; XENOPUS LAEVIS;

ANIMALIA; ORYCTOLAGUS CUNICULUS; XENOPUS LAEVIS;

EID: 0035864826     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/10.4.329     Document Type: Article

References (31)

1. Epidemiology
2. Understanding the molecular basis of fragile X syndrome
3. Tissue specific expression of FMR-1 provides evidence for a functional role in fragile X syndrome
4. Human and murine FMR1: Alternative splicing and translational initiation downstream of the CGG-repeat
5. FXR1, an autosomal homolog of the fragile X mental retardation gene
6. Alternative splicing in the fragile X gene FMR1
7. The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein
8. FMR1 protein: Conserved RNP family domains and selective RNA binding
9. Purified recombinant Fmrp exhibits selective RNA binding as an intrinsic property of the fragile X mental retardation protein
10. The fragile X mental retardation syndrome protein interacts with novel homologs FXR1 and FXR2
11. A point mutation in the FMR1 gene associated with fragile X mental retardation
12. Essential role for KH domains in RNA binding: Impaired RNA binding by a mutation in the KH domain of FMR1 that causes fragile X syndrome
13. FMRP associates with polyribosomes as an mRNP, and the 1304N mutation of severe fragile X syndrome abolishes this association
14. Different targets in the fragile X-related proteins revealed by their distinct nuclear localizations
15. A nuclear role for the Fragile X mental retardation protein
16. The fragile X mental retardation protein is a ribonucleoprotein containing both nuclear localization and nuclear export signals
17. Molecular characterization of the protein products of the fragile X syndrome gene and the survival of motor neurons gene
18. Analysis of domains affecting intracellular localization of the FMRP protein
19. The fragile X-related proteins FXR1P and FXR2P contain a functional nucleolar-targeting signal equivalent to the HIV-1 regulatory proteins
20. FMRP is associated with the ribosomes via RNA
21. + mRNA in actively translating polyribosomes
22. Specific sequences in the fragile X syndrome protein FMR1 and the FXR protein mediate their binding to 60S ribosomal subunits and the interactions among them
23. Isolation of an FMRP-associated messenger ribonucleoprotein particle and identification of nucleolin and the fragile X-related proteins as components of the complex
24. From factors to mechanisms: Translation and translational control in eukaryotes
25. Dissecting FMR1, the protein responsible for fragile X syndrome, in its structural and functional domains
26. SMN oligomerization defect correlates with spinal muscular atrophy severity
27. SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins
28. (Over)correction of FMR1 deficiency with YAC transgenics: Behavioral and physical features
29. Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: Implications for spinal muscular atrophy
30. mRNA silencing in erythroid differentiation: hnRNP K and hnRNP E1 regulate 15-lipoxygenase translation from the 3′ end
31. The HIV-1 Rev activation domain is a nuclear export signal that accesses an export pathway used by specific cellular RNAs