. Author manuscript; available in PMC: 2016 Jul 6.

Published in final edited form as: Integr Biol (Camb). 2015 Jul;7(7):758–775. doi: 10.1039/c5ib00031a

Table 1. Characteristics of protein-aggregating neurodegenerative diseases.

	Primary constituent of protein aggregation	Location of Aggregates	Commonly Studied Familial Genes	Familial Forms (% of total disease count)	Primary Region of Neuronal Loss	Average age at onset
AD	Aβ, tau	extracellular	PSEN1, PSEN2, APP, APOE³²	< 5%³²	entorhinal cortex, hippocampus	70^33–35
FTD	ubiquitin, TDP-43, FUS, tau	cytoplasmic (neuronal and glial), intranuclear (neuronal)	TARDBP, FUS, C9ORF72, PGRN VCP, MAPT³⁶	25-50%^32,37,38	frontal and/or temporal lobe	60^39,40
ALS	ubiquitin, SOD1, TDP-43, FUS	cytoplasmic	SOD1, C9ORF72, FUS, TARDBP, OPTN^41–43	5-10%^32,44	motor cortex, brainstem, spinal cord	60⁴⁵
PD	α-synuclein	cytoplasmic	SNCA, LRRK2, PINK1, PRKN, DJ1³²	<10%⁴⁶	substantia nigra	60³³
HD	htt	intranuclear and cytoplasmic	HTT⁴⁷	∼90%³²	striatum	^*

Dependent on number of CAG repeats.⁴⁸