Table 1.

TDP-43 in neurodegenerative disorders adapted from [15, 50, 62, 71]

Pathologic classification	Clinical presentation	Genetic links	TDP-43 cellular locations	Frequency in disease
FTLD-TDP type 1	FTDbv or PNFA	Progranulin (RGN) mutations	DN, NCIs and NII in layer II of frontal and temporal cortex.	32% of FTLD-TDP43
FTLD-TDP type 2	Semantic dementia	None	DN predominant in lower layers of cerebral cortex	27% of FTLD-TDP43
FTLD-TDP type 3	Motor neuron disease	Familial cases linked to chromosome 9	NCI in cerebral cortex and hippocampus	42% of FTLD-TDP43
AD	Unknown	Unknown	DN and NCI in amygdala and hippocampus	23%
Normal	N/A	Unknown	DN, NCI and NII in amygdala and hippocampus	3%

Classification according to Mackenzie, et al. [72] Abbreviations: dystrophic neurites (DN), neuronal cytoplasmic inclusions (NCI), intraneuronal inclusions (NII), progressive non-fluent aphasia (PNFA), FTDbv = behavioral variant of frontotemporal dementia; N/A = not appropriate.