Table 1.

Frequency and morphology of cellular inclusions in ALS-FUS

	BI	BI	BI	FUS-ir	FUS-ir	FUS-ir	FUS-ir	FUS-ir
				NCI	NCI	NCI	NCI	GCI
	Motor cortex	Subcort	Lower motor neurons	Motor cortex	Subcort	Lower motor neurons	Morph
Early onset, short duration	++	+	++	+++	++a	+++	Round	+
Late onset, long duration	−	−	+	+	++b	+++	Tangles	+++

Based on data in study by Mackenzie et al. 2011a.

Grading: +, few; ++, moderate; +++, numerous.

ALS-FUS, Amyotrophic lateral sclerosis caused by FUS mutations; BI, basophilic inclusion; ir, immunoreactive; NCI, neuronal cytoplasmic inclusion; GCI, glial cytoplasmic inclusion.

^aNucleus of the basis pontis, cerebellar dentate nucleus, and substantia nigra.

^bBasal ganglia, thalamus, and substantia nigra.