Table 3.

Functional consequences of DNAJB6 mutations.

Protein Change	PolyQ Aggregation	PARK p.C289G Aggregation	Sis1 Complementation 1	[RNQ+] Propagation 2	[PSI+] Propagation/Solubility 2	TDP-43 Aggregation	hnRNPA2 p.D290V Aggregation 3	Hrb98DE Localization & interaction 3	Myotoxicity in Zebrafish	References
A50V	†††††					†				[90]
E54A	††††					†				[90]
F89I	††††		††	††/†	††	†	†	†	†††	[14,59,105]
F91I	††									[95]
F91L	†††								†††	[95]
F93L	†	†	–	–	–	†	†		††(†)	[14,31,59,90,105]
N95I	††††									[89]
P96R	†††††		†††	†/–	†	†			†	[105], this paper
P96L	†††									[102]
D98del	††††									[89]
F100I	†††††									this paper

Number of † symbols indicates severity of observed defect, – no defect observed, (empty) not reported. Results based on corresponding mutations in: ¹ DNAJB1, ² chimeric Sis1/DNAJB6 construct, ³ Drosophila dMRJ.