Accordingly, Hsp110 should be explored further as a potential therapeutic target to decrease the degradation of ASPA and other misfolded proteins, possibly using a recently developed inhibitor of Hsp110 [81], to potentially avoid or diminish the development of Canavan disease and other protein misfolding disorders such as Lynch syndrome or Birt-Hogg-Dubé syndrome [82,83]. Here, ASPA is linked to Lynch syndrome.