8.A.192. The DnaJ Homolog (DnaJ) Family
The DnaJ family members are found in several TC families: 1.P.1, 3.A.16, 3.A.30 and 3.A.5. These proteins are chaparone proteins that often function with DnaK (Barriot et al. 2020). Mutations in DNAJC5/CSPalpha are associated with adult neuronal ceroid lipofuscinosis (ANCL), a dominant-inherited neurodegenerative disease featuring lysosome-derived autofluorescent storage materials (AFSMs) termed lipofuscin. Functionally, DnaJC5 has been implicated in chaperoning synaptic proteins and in misfolding-associated protein secretion (Lee et al. 2022).