Table 1.

Genes for motor neuron disease with upper motor neuron involvement, the mouse models generated, and the investigation of the cortical component of motor neuron circuitry.

Disease	Gene	Mouse Model Available	Motor Cortex Involvement
SPG5A	CYP7B1 [34,35]	Y [36,37]
SPG7	PARAPLEGIN [38,39,40,41,42]	Y [43,44,45]
SPG11	SPATACSIN [46]	Y [47,48]	Y [47,48]
SPG15	ZFYVE26 (SPASTIZIN) [49]	Y [50]	Y [50]
SPG20	SPARTIN [51,52]	Y [53]
SPG21	MASPARDIN [54]	Y [25]	N [25]
SPG26	B4GALNT1 (GM1, GALNACT) [55]	Y [23]	N [23]
SPG28	DDHD1 (PAPLA) [56]	Y [57,58]
SPG30	KIF1A [59]	Y [60,61,62]
SPG35	FA2H [63]	Y [64,65]
SPG39	PNPLA6 (NTE) [66]	Y [26,67]	N [26]
SPG44	GJC2 (CX47) [68]	Y [69,70,71,72]
SPG45	NT5C2 [73]	Y [74]
SPG46	GBA2 [75,76]	Y [77,78,79,80]
SPG47	AP-4 [81]	Y [82]
SPG48	KIAA0415 (AP-5Z1) [83]	Y [84]	Y [84]
SPG54	DDHD2 (KIAA0725P, IPLA1γ) [85]	Y [86,87]
SPG63	AMPD2 [88]	Y [89,90,91]
SPG64	ENTPD1 (CD39) [88]	Y [92,93,94]
SPG75	MAG [88,95]	Y [96,97,98,99,100,101,102,103]
SPG76	CAPN1 [104]	Y [105,106,107,108]
SPG78	ATP13A2 [109,110]	Y [111,112,113,114,115,116,117,118]
SPG79	UCHL1 [119]	Y [120,121,122,123,124,125,126,127]	Y [120,124,125,128,129]
SPG3A	ATL1 [130,131,132]	Y [133,134]
SPG4	SPAST [135,136]	Y [137,138,139,140]	Y [140]
SPG6	NIPA1 (CXFIP1) [141]	Y [142]
SPG8	KIAA0196 (WASH C5, STRUMPELLIN, RTSC1) [143,144,145]	Y [146]
SPG10	KIF5A [147]	Y [24,148]	N [24]
SPG12	RTN2 (NSPL1) [149]	Y [150]
SPG13	SSPD1 (HSP60, HSPD1) [151]	Y [152,153,154,155]	Y [153,155]
SPG17	BSCL2/SEIPIN [156,157]	Y [27,158,159,160]	N [27]
SPG31	REEP1 [161,162]	Y [163,164,165]	Y [163,165]
SPG42	SLC33A1 [166,167]	Y [168]
SPG73	CPT1C [169]	Y [170,171]	Y [170]
SPG1	L1CAM [172]	Y [173,174,175]	Y [173,174]
SPG2	PLP1 [176,177,178,179]	Y [176,180,181,182,183,184,185,186,187]
PLS	ALS2 [188,189,190,191,192]	Y [28,193,194,195,196,197]	Y [28]