Table 3.

Genetic correlates of the neuropathological subtypes of frontotemporal lobar degeneration (FTLD)^a

Molecular classification	Neuropathological subtypeb	Mutated genesb
FTLD-tau	Pick’s disease	MAPT
	Corticobasal degeneration	MAPT
	Progressive supranuclear palsy	MAPT
	Argyrophilic grain disease	MAPT
	Neurofibrillary tangle–predominant dementia	MAPT
	Multiple system tauopathy with dementia	MAPT
	White matter tauopathy with globular glial inclusions	MAPT
FTLD–TAR DNA-binding protein 43	Type A	GRN
		C9orf72
	Type B	C9orf72
	Type C
	Type D	VCP
FTLD–fused in sarcoma	Atypical FTLD with ubiquitinated inclusions	FUS
	Neuronal intermediate filament inclusion disease	FUS
	Basophilic inclusion body disease	FUS
FTLD–ubiquitin proteasome system	Frontotemporal dementia linked to chromosome 3	CHMP2B

^aAdapted from Reference 154.

^bCharacteristic pattern of pathology, not the clinical syndrome.