Mouse models of the fragile x premutation and the fragile X associated tremor/ataxia syndrome

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Mouse models of the fragile x premutation and the fragile X associated tremor/ataxia syndrome is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1007/978-3-642-21649-7_14
P932PMC publication ID4313770
P698PubMed publication ID22009357

P50authorMichael R. HunsakerQ46157201
P2093author name stringRobert F Berman
Rob Willemsen
Michael R Hunsaker
Renate K Hukema
Gloria Arque
P2860cites workThe fragile-X premutation: a maturing perspectiveQ24533443
Penetrance of the fragile X-associated tremor/ataxia syndrome in a premutation carrier populationQ28241139
The FMR1 CGG repeat mouse displays ubiquitin-positive intranuclear neuronal inclusions; implications for the cerebellar tremor/ataxia syndromeQ28507736
Cognitive decline, neuromotor and behavioural disturbances in a mouse model for fragile-X-associated tremor/ataxia syndrome (FXTAS).Q30988960
Covariate adjusted correlation analysis with application to FMR1 premutation female carrier dataQ33403351
Lifetime prevalence of mood and anxiety disorders in fragile X premutation carriersQ33684243
Advances in understanding the molecular basis of FXTASQ33871325
The primary cognitive deficit among males with fragile X-associated tremor/ataxia syndrome (FXTAS) is a dysexecutive syndrome.Q33901895
Fragile X premutation tremor/ataxia syndrome: molecular, clinical, and neuroimaging correlatesQ33905067
Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation.Q33987114
Elevated levels of FMR1 mRNA in carrier males: a new mechanism of involvement in the fragile-X syndrome.Q34146206
Lifespan changes in working memory in fragile X premutation malesQ34154343
Potassium bromate, a potent DNA oxidizing agent, exacerbates germline repeat expansion in a fragile X premutation mouse modelQ34182956
Premutation CGG-repeat expansion of the Fmr1 gene impairs mouse neocortical development.Q34399389
FMR1 CGG repeat length predicts motor dysfunction in premutation carriers.Q34721602
Clinical and neuropathologic findings in a woman with the FMR1 premutation and multiple sclerosisQ34840680
Ubiquitin-positive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutationQ34915612
The FMR1 gene and fragile X-associated tremor/ataxia syndromeQ35061671
A mouse model of the fragile X premutation: effects on behavior, dendrite morphology, and regional rates of cerebral protein synthesisQ35148962
Altered hypothalamus-pituitary-adrenal gland axis regulation in the expanded CGG-repeat mouse model for fragile X-associated tremor/ataxia syndromeQ35533280
Expression of the FMR1 geneQ35550083
Elevated FMR1 mRNA in premutation carriers is due to increased transcription.Q35697473
Elevated Fmr1 mRNA levels and reduced protein expression in a mouse model with an unmethylated Fragile X full mutationQ35751997
Regional FMRP deficits and large repeat expansions into the full mutation range in a new Fragile X premutation mouse modelQ35944748
ATR protects the genome against CGG.CCG-repeat expansion in Fragile X premutation miceQ36457201
CGG-repeat length and neuropathological and molecular correlates in a mouse model for fragile X-associated tremor/ataxia syndrome.Q37021134
The fragile X continuum: new advances and perspectivesQ37150380
Ectopic expression of CGG containing mRNA is neurotoxic in mammalsQ37223278
The fragile X prevalence paradoxQ37310009
ATM and ATR protect the genome against two different types of tandem repeat instability in Fragile X premutation miceQ37406053
Molecular pathogenesis of fragile X-associated tremor/ataxia syndromeQ37455479
Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degenerationQ37471792
Early onset of neurological symptoms in fragile X premutation carriers exposed to neurotoxinsQ37569365
Long uninterrupted CGG repeats within the first exon of the human FMR1 gene are not intrinsically unstable in transgenic miceQ38335797
Motor cortex stroke impairs individual digit movement in skilled reaching by the rat.Q39857278
Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patientsQ40117267
Human topological task adapted for rats: Spatial information processes of the parietal cortexQ41870313
Progressive spatial processing deficits in a mouse model of the fragile X premutationQ42119514
The ladder rung walking task: a scoring system and its practical applicationQ42949417
Intranuclear inclusions in neural cells with premutation alleles in fragile X associated tremor/ataxia syndromeQ43073775
Cortical and subcortical lesions impair skilled walking in the ladder rung walking test: a new task to evaluate fore- and hindlimb stepping, placing, and co-ordinationQ43981348
Absence of impairments or recovery mediated by the uncrossed pyramidal tract in the rat versus enduring deficits produced by the crossed pyramidal tractQ44111452
FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome (FXTAS).Q45976696
Stepping test in mice: a reliable approach in determining forelimb akinesia in MPTP-induced Parkinsonism.Q46002034
Neuropathology of fragile X-associated tremor/ataxia syndrome (FXTAS).Q46007399
Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriersQ46124789
Expression of the GABAergic system in animal models for fragile X syndrome and fragile X associated tremor/ataxia syndrome (FXTAS).Q46205774
Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X.Q46666276
Testicular and pituitary inclusion formation in fragile X associated tremor/ataxia syndromeQ46723998
Screen for excess FMR1 premutation alleles among males with parkinsonismQ46724443
Lesions to the ventral, but not the dorsal, medial prefrontal cortex enhance latent inhibition.Q48239833
Hand shaping in the rat: conserved release and collection vs. flexible manipulation in overground walking, ladder rung walking, cylinder exploration, and skilled reachingQ48259762
Inactivation of the prelimbic, but not infralimbic, prefrontal cortex impairs the contextual control of response conflict in ratsQ48281436
Instability of CGG repeats in transgenic miceQ48289415
Progression of tremor and ataxia in male carriers of the FMR1 premutationQ48354732
Dissociating the role of the parietal cortex and dorsal hippocampus for spatial information processingQ48466605
Quantitative and qualitative impairments in skilled reaching in the mouse (Mus musculus) after a focal motor cortex strokeQ48557223
Deficits in response initiation, but not attention, following excitotoxic lesions of posterior parietal cortex in the rat.Q48557780
Trinucleotide repeats (CGG)22TGG(CGG)43TGG(CGG)21 from the fragile X gene remain stable in transgenic miceQ48952707
The emerging fragile X premutation phenotype: evidence from the domain of social cognition.Q50982662
Age-dependent cognitive changes in carriers of the fragile X syndrome.Q51890929
Contextual control of biconditional task performance: evidence for cue and response competition in rats.Q51944601
Reduced FMRP and increased FMR1 transcription is proportionally associated with CGG repeat number in intermediate-length and premutation carriers.Q51965229
Fragile X males with unmethylated, full mutation trinucleotide repeat expansions have elevated levels of FMR1 messenger RNA.Q51973841
FMR1 premutation allele (CGG)81 is stable in mice.Q51997531
Clinical involvement and protein expression in individuals with the FMR1 premutation.Q52169510
Instability of a (CGG)98 repeat in the Fmr1 promoter.Q52543579
Bilateral alteration in stepping pattern after unilateral motor cortex injury: A new test strategy for analysis of skilled limb movements in neurological mouse modelsQ61695772
Covert orienting of attention in the rat and the role of striatal dopamineQ71059959
P921main subjectfragile X-associated tremor/ataxia syndromeQ1440436
P304page(s)255-269
P577publication date2012-01-01
P1433published inResults and problems in cell differentiationQ26842363
P1476titleMouse models of the fragile x premutation and the fragile X associated tremor/ataxia syndrome
P478volume54

Reverse relations

cites work (P2860)
Q37528664Abnormal semantic processing in females with fragile X-associated tremor/ataxia syndrome.
Q34295112Cerebral protein synthesis in a knockin mouse model of the fragile X premutation
Q36048562Current Gaps in Understanding the Molecular Basis of FXTAS
Q37524454Fragile X-associated tremor/ataxia syndrome (FXTAS): pathology and mechanisms
Q90699685In silico, in vitro, and in vivo Approaches to Identify Molecular Players in Fragile X Tremor and Ataxia Syndrome
Q36188438Induced expression of expanded CGG RNA causes mitochondrial dysfunction in vivo
Q34055453Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome
Q37623407Neurocognitive endophenotypes in CGG KI and Fmr1 KO mouse models of Fragile X-Associated disorders: an analysis of the state of the field
Q30008925Premutation in the Fragile X Mental Retardation 1 (FMR1) Gene Affects Maternal Zn-milk and Perinatal Brain Bioenergetics and Scaffolding
Q34243144RNA-protein interactions in unstable microsatellite diseases.
Q37663611Reduced activity-dependent protein levels in a mouse model of the fragile X premutation.
Q28392778Using mouse models of autism spectrum disorders to study the neurotoxicology of gene-environment interactions

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