Table 1.

Examples of the function of neuronal splicing regulators in neuronal differentiation and brain development.

Splicing regulators	Targets	Knockout/downregulation of splicing regulators		References
		Target exon	Phenotypes
nSR100/SRRM4	Protrudin (Zfyve27)	suppressed inclusion of exon L between exon 8 and 9	impaired neurite outgrowth	Ohnishi et al., 2017
Ptbp1 & 2	PSD-95 (Dlg4)	exon 18 inclusion	impaired development of glutamatergic neurons	Zheng et al., 2012
Ptbp1	Flna	included the poison exon	brain specific malformation	Zhang et al., 2016
Ptbp2	Dnm1	altered mutually exclusive selection of exons 9a/9b	impaired synaptic function, and caused seizures and behavioral deficits	Li et al., 2014
Nova2	Dab1	exon 7bc (9bc) inclusion	impaired radial migration and Purkinje neuron migration	Yano et al., 2010
Rbfox3	Numb	repressed Numb exon 12 inclusion	impaired neuronal differentiation	Kim et al., 2013
Rbfox1	Snap25	altered mutually exclusive selection of exons 5a/5b	caused seizure	Gehman et al., 2011
SRSF1	ApoER2	promote exon 19 inclusion	impaired synapse formation and function	Hinrich et al., 2016
hnRNP H1/H2	TRF2	exon 7 (TRF2-S) inclusion	impaired neuronal differentiation.	Grammatikakis et al., 2016
RBM4	Numb	increased exon 3 skipping and exon 9 inclusion	impaired neuronal differentiation and neuronal outgrowth	Tarn et al., 2016

PSD-95: postsynaptic density protein 95.

Dnm1: dynamin1.

Flna: filamin A.

Dab1: disabled homolog-1.

hnRNP: heterogeneous nuclear ribonucleoprotein.

Snap25: synaptosomal-associated protein 25.

ApoER2: apolipoprotein E receptor 2.

TRF2: telomeric repeat-binding factor 2.