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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).

References associated with 8.A.192 family:

Barriot, R., J. Latour, M.P. Castanié-Cornet, G. Fichant, and P. Genevaux. (2020). J-Domain Proteins in Bacteria and Their Viruses. J. Mol. Biol. 432: 3771-3789. 32305462
Boal, F., M. Laguerre, A. Milochau, J. Lang, and P.A. Scotti. (2011). A charged prominence in the linker domain of the cysteine-string protein Cspα mediates its regulated interaction with the calcium sensor synaptotagmin 9 during exocytosis. FASEB J. 25: 132-143. 20847230
Lee, J., Y. Xu, L. Saidi, M. Xu, K. Zinsmaier, and Y. Ye. (2022). Abnormal triaging of misfolded proteins by adult neuronal ceroid lipofuscinosis-associated DNAJC5/CSPα mutants causes lipofuscin accumulation. Autophagy 1-20. [Epub: Ahead of Print] 35506243
Sharma, M., J. Burré, P. Bronk, Y. Zhang, W. Xu, and T.C. Südhof. (2012). CSPα knockout causes neurodegeneration by impairing SNAP-25 function. EMBO. J. 31: 829-841. 22187053