Elevated Fmr1 mRNA levels and reduced protein expression in a mouse model with an unmethylated Fragile X full mutation.
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Molecular Correlates and Recent Advancements in the Diagnosis and Screening of FMR1-Related DisordersDetection and Quantification of the Fragile X Mental Retardation Protein 1 (FMRP)Modeling Fragile X Syndrome Using Human Pluripotent Stem CellsFrom FMRP function to potential therapies for fragile X syndrome.Comprehensive neurocognitive endophenotyping strategies for mouse models of genetic disordersCGG repeat in the FMR1 gene: size mattersMouse models of fragile X-associated tremor ataxia.Fibroblast phenotype in male carriers of FMR1 premutation allelesCNS expression of murine fragile X protein (FMRP) as a function of CGG-repeat size.Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.iPSC-derived forebrain neurons from FXS individuals show defects in initial neurite outgrowthMouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndromeExpression of an expanded CGG-repeat RNA in a single pair of primary sensory neurons impairs olfactory adaptation in Caenorhabditis elegans.Clinical and molecular implications of mosaicism in FMR1 full mutations.Cerebral protein synthesis in a knockin mouse model of the fragile X premutation.Premutation CGG-repeat expansion of the Fmr1 gene impairs mouse neocortical development.Promoter-bound trinucleotide repeat mRNA drives epigenetic silencing in fragile X syndrome.Ubiquitin-positive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation.Mouse models of the fragile x premutation and the fragile X associated tremor/ataxia syndrome.The FMR1 gene and fragile X-associated tremor/ataxia syndrome.Microsatellite repeat instability and neurological disease.A mouse model of the fragile X premutation: effects on behavior, dendrite morphology, and regional rates of cerebral protein synthesis.The fragile x mental retardation syndrome 20 years after the FMR1 gene discovery: an expanding universe of knowledge.Altered hypothalamus-pituitary-adrenal gland axis regulation in the expanded CGG-repeat mouse model for fragile X-associated tremor/ataxia syndrome.Widespread non-central nervous system organ pathology in fragile X premutation carriers with fragile X-associated tremor/ataxia syndrome and CGG knock-in mice.FMR1 premutation and full mutation molecular mechanisms related to autism.Regional FMRP deficits and large repeat expansions into the full mutation range in a new Fragile X premutation mouse modelClustered burst firing in FMR1 premutation hippocampal neurons: amelioration with allopregnanolone.Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivoPresence of inclusions positive for polyglycine containing protein, FMRpolyG, indicates that repeat-associated non-AUG translation plays a role in fragile X-associated primary ovarian insufficiency.Early mitochondrial abnormalities in hippocampal neurons cultured from Fmr1 pre-mutation mouse model.Distribution and frequency of intranuclear inclusions in female CGG KI mice modeling the fragile X premutationMonogenic mouse models of autism spectrum disorders: Common mechanisms and missing links.Enhanced asynchronous Ca(2+) oscillations associated with impaired glutamate transport in cortical astrocytes expressing Fmr1 gene premutation expansionEpigenetics in nucleotide repeat expansion disorders.Dnmt1 deficiency promotes CAG repeat expansion in the mouse germlineGranulosa cell and oocyte mitochondrial abnormalities in a mouse model of fragile X primary ovarian insufficiency.CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome.The cyclic AMP phenotype of fragile X and autismThe quadruplex r(CGG)n destabilizing cationic porphyrin TMPyP4 cooperates with hnRNPs to increase the translation efficiency of fragile X premutation mRNA
P2860
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P2860
Elevated Fmr1 mRNA levels and reduced protein expression in a mouse model with an unmethylated Fragile X full mutation.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@ast
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@en
type
label
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@ast
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@en
prefLabel
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@ast
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@en
P2093
P2860
P1476
Elevated Fmr1 mRNA levels and ...... lated Fragile X full mutation.
@en
P2093
B A Oostra
C E Bakker
D L Nelson
E J Mientjes
H C Van der Linde
I M Nieuwenhuizen
J R Brouwer
L A Severijnen
R Willemsen
P2860
P304
P356
10.1016/J.YEXCR.2006.10.002
P407
P577
2006-10-13T00:00:00Z