Regulation of molecular pathways in the Fragile X Syndrome: insights into Autism Spectrum Disorders

Table 1 FMRP functions and target mRNAs

FMRP Function	Regulation	Target MRNAS	Where FMRP affects their regulation	How FMRP affects their regulation	References
Stability	+	PSD-95	Mouse hippocampus	Activity-dependent	Zalfa et al. 2007
Stability	+	GABA subunits	Mouse cortex; Drosophila.	mRNA levels are reduced but a direct evidence on their different stability is not available	Gantois et al. 2006; D’Hulst et al. 2006; Dictenberg et al. 2008
Transport	+	Map1B	Primary hippocampal neurons	Activity-driven transport	Dictenberg et al. 2008
	+	α-CaMKII	Primary hippocampal neuron	Activity-driven transport	Dictenberg et al. 2008; Kao et al. 2010
	+	Sapap4	Primary hippocampal neurons	Activity-driven transport	Dictenberg et al. 2008
	+	Rgs5	Hippocampal slices	Basal mRNA localization	Miyashiro et al. 2003
Translation	−	Map1B	Mouse brain and hippocampal slices; human FXS lymphoblastoid cells;	Basal and activity-dependent	Brown et al. 2001; Zalfa et al. 2003; Lu et al. 2004; Hou et al. 2006
	−	Arc	Mouse brain; hippocampal slices; synaptoneurosomes	Basal and activity-dependent	Zalfa et al. 2003; Park et al. 2008
	−	α-CaMKII	Mouse brain; hippocampal slices; synaptoneurosomes	Basal and activity-dependent	Zalfa et al. 2003; Hou et al. 2006; Muddashetty et al. 2007; Kao et al. 2010
	−	App	Cortical synaptoneurosomes	Basal and activity-dependent; antagonistic effects with HnRNPC	Westmark and Malter 2007; Lee et al. 2010
	−	PSD-95	Cortical synaptoneurosomes	Activity-dependent	Todd et al. 2003; Muddashetty et al. 2007

Three well-characterized FMRP functions, namely mRNA stability, transport and translation, are indicated. Established FMRP target mRNAs are listed. Other studies on additional putative mRNA targets are cited in the text

ISSN: 1866-1955