Topic: fragile X mental retardation protein

Fragile X Syndrome (FXS), a frequent form of inherited intellectual disability and a leading monogenic cause of autism, is caused by lack/reduction of fragile X mental retardation protein (FMRP), an RNA-binding protein involved in several aspects of RNA metabolism. Abnormal expression of proteins at synapses underlies brain dysfunction in FXS. FMRP is also implicated in DNA damage response and is a component of stress granules (SGs), cytoplasmatic aggregates that form in response to stress and are protective against apoptosis. Evidence in the mouse model of FXS suggests an excess of oxidizing

Kinesin 1 (KIF5) is one of the major types of motor proteins that transport synaptic proteins and mRNAs to dendrites, but their function in dendritic transport of neurons in vivo remains undefined. Using in vivo two-photon longitudinal imaging, we found that mice with conditional knockout of kif5b (KIF5B cKO) in CaMKIIα-Cre expressing neurons showed heightened turnover and lower stability of dendritic spines in L2/3 pyramidal neurons in the frontal association cortex. The increase of dendritic spine instability was accompanied with less postsynaptic density 95 (PSD95) acquisition in dendritic