Unknown

Dataset Information

0

Neuropeptide Release is Impaired in a Mouse Model of Fragile X Mental Retardation Syndrome.

ABSTRACT: Fragile X syndrome (FXS), an inherited disorder characterized by mental retardation and autismlike behaviors, is caused by the failure to transcribe the gene for fragile X mental retardation protein (FMRP), a translational regulator and transporter of select mRNAs. FXS model mice (Fmr1 KO mice) exhibit impaired neuropeptide release. Release of biogenic amines does not differ between wild-type (WT) and Fmr1 KO mice. Rab3A, an mRNA cargo of FMRP involved in the recruitment of vesicles, is decreased by ∼50% in synaptoneurosomes of Fmr1 KO mice; however, the number of dense-core vesicles (DCVs) does not differ between WT and Fmr1 KO mice. Therefore, deficits associated with FXS may reflect this aberrant vesicle release, specifically involving docking and fusion of peptidergic DCVs, and may lead to defective maturation/maintenance of synaptic connections.

SUBMITTER: Annangudi SP 

PROVIDER: S-EPMC2873207 | biostudies-literature | 2010 Jan

REPOSITORIES: biostudies-literature

Json Xml
altmetric image

Publications

Fragile X syndrome (FXS), an inherited disorder characterized by mental retardation and autismlike behaviors, is caused by the failure to transcribe the gene for fragile X mental retardation protein (FMRP), a translational regulator and transporter of select mRNAs. FXS model mice (Fmr1 KO mice) exhibit impaired neuropeptide release. Release of biogenic amines does not differ between wild-type (WT) and Fmr1 KO mice. Rab3A, an mRNA cargo of FMRP involved in the recruitment of vesicles, is decrease  ...[more]

Similar Datasets

Unknown Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome.
| S-EPMC2992329 | biostudies-other
Unknown SIRT1 inhibition alleviates gene silencing in Fragile X mental retardation syndrome.
| S-EPMC2265469 | biostudies-literature
Unknown Impaired synaptic incorporation of AMPA receptors in a mouse model of fragile X syndrome
| S-EPMC10665894 | biostudies-literature
Unknown Cellular distribution of the fragile X mental retardation protein in the mouse brain.
| S-EPMC5558202 | biostudies-other
Unknown Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP).
| S-EPMC2773141 | biostudies-literature
Unknown Impaired hippocampal representation of place in the Fmr1-knockout mouse model of fragile X syndrome.
| S-EPMC5995880 | biostudies-literature
Unknown CUL4B ubiquitin ligase in mouse development: a model for human X-linked mental retardation syndrome?
| S-EPMC3411174 | biostudies-other
Other The Fragile X Mental Retardation Protein (FMRP) controls diacylglycerol kinase in neurons
2016-06-01 | GSE51649 | GEO
Transcriptomics The Fragile X Mental Retardation Protein (FMRP) controls diacylglycerol kinase in neurons
2016-06-01 | E-GEOD-51649 | biostudies-arrayexpress
Unknown The prognostic significance of fragile X mental retardation syndrome-related protein 1 (FXR1) in breast cancer.
| S-EPMC11912039 | biostudies-literature