TCDB is operated by the Saier Lab Bioinformatics Group

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
Drwesh, L., B. Heim, M. Graf, L. Kehr, L. Hansen-Palmus, M. Franz-Wachtel, B. Macek, H. Kalbacher, J. Buchner, and D. Rapaport. (2022). A network of cytosolic (co)chaperones promotes the biogenesis of mitochondrial signal-anchored outer membrane proteins. Elife 11:. 35876647
Fujibayashi, A., T. Taguchi, R. Misaki, M. Ohtani, N. Dohmae, K. Takio, M. Yamada, J. Gu, M. Yamakami, M. Fukuda, S. Waguri, Y. Uchiyama, T. Yoshimori, and K. Sekiguchi. (2008). Human RME-8 is involved in membrane trafficking through early endosomes. Cell Struct Funct 33: 35-50. 18256511
Girard, M. and P.S. McPherson. (2008). RME-8 regulates trafficking of the epidermal growth factor receptor. FEBS Lett. 582: 961-966. 18307993
Hon, T., H.C. Lee, A. Hach, J.L. Johnson, E.A. Craig, H. Erdjument-Bromage, P. Tempst, and L. Zhang. (2001). The Hsp70-Ydj1 molecular chaperone represses the activity of the heme activator protein Hap1 in the absence of heme. Mol. Cell Biol. 21: 7923-7932. 11689685
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
Norris, A., C.T. McManus, S. Wang, R. Ying, and B.D. Grant. (2022). Mutagenesis and structural modeling implicate RME-8 IWN domains as conformational control points. PLoS Genet 18: e1010296. 36279308
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