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Gene Review

DNAJB1P1  -  DnaJ (Hsp40) homolog, subfamily B, member...

Homo sapiens

Synonyms: DNAJB1P, HSP40, psiHSP40
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Disease relevance of HSP40

  • Twenty-six tissue specimens of inflamed areas from patients with UC, 16 from patients with Crohn's disease (CD) and 16 endoscopically normal tissues were analysed for HSP40 expression [1].
  • In patients with inactive disease, those with proctitis or left-sided colitis had higher titres of anti-HSP40 autoantibodies than those with total colitis [1].
  • Autoantibodies against heat shock protein 40 (HSP40) and their clinical significance in ulcerative colitis (UC) have not been evaluated before [1].
  • A significative correlation between the detection of human viruses by PCR and the values of bacteriophages of B. fragilis HSP40 in urban raw sewage was observed [2].
  • Bacteroides fragilis HSP40 phages have been detected in waters with various levels of fecal contamination of human origin [3].

High impact information on HSP40

  • Cysteine string protein (CSP) alpha is an abundant synaptic vesicle protein that contains a DNA-J domain characteristic of Hsp40-type cochaperones [4].
  • The cluster of genes whose up-regulation was potentiated by GSH depletion included two HSPs (HSP40 and HSP70) and the AP-1 transcription factor components Fos and FosB [5].
  • Such overproduction of Hap46 also resulted in enhanced expression of specific reporter gene constructs and in increased levels of mRNAs specific for hsp70 and hsp40 after temperature stress [6].
  • Heat shock proteins Hsp70 and Hsp40 co-localize with the aggregates and activation of the chaperone system by geldanamycin leads to a reduction of aggregate formation [7].
  • Dual function of membrane-bound heat shock protein 70 (Hsp70), Bag-4, and Hsp40: protection against radiation-induced effects and target structure for natural killer cells [8].

Chemical compound and disease context of HSP40


Biological context of HSP40


Anatomical context of HSP40


Associations of HSP40 with chemical compounds

  • In eukaryotes, newly synthesized proteins interact co-translationally with a multitude of different ribosome-bound factors and chaperones including the conserved heterodimeric nascent polypeptide-associated complex (NAC) and a Hsp40/70-based chaperone system [18].
  • Some chaperones such as Hsp40 and Hsp70 have been identified as important regulators of polyglutamine aggregation and/or cell death in neuronal cells [19].
  • In addition, several heat-shock proteins (Hsp) [Hsp27 (2.78-fold), Hsp70 (3.75-fold), and Hsp40-like protein (3.21-fold)] were induced by curcumin [20].
  • 17-AAG induced Hsp70 and Hsp40 in vivo as previously reported; however, its ability to induce HSPs was limited, suggesting that the HSP induction might support the degradation of mutant protein [21].
  • Domain requirements of DnaJ-like (Hsp40) molecular chaperones in the activation of a steroid hormone receptor [22].

Physical interactions of HSP40


Regulatory relationships of HSP40

  • In contrast, three compounds inhibited the ability of Hsp40 to stimulate Hsp70 ATPase activity but did not affect the endogenous activity of Hsp70 [24].

Other interactions of HSP40

  • Two of these agents also compromised the Hsp70/Hsp40-mediated post-translational translocation of a secreted pre-protein in vitro [24].
  • Some genes induced by OS in the current study (e.g., oxygen regulated protein [ORP150] and heat shock protein [HSP40]) are better known to respond to other forms of stress [25].
  • Furthermore, Cdc48 can act as a co-chaperone in the Hsc70-Hsp40 chaperone system [26].

Analytical, diagnostic and therapeutic context of HSP40

  • The expression of six SPs--MT, HSP25/27, HSP40, HSP60, HSP70, and HSP90--was then determined by ELISA [27].
  • Immunohistochemistry showed that 17 out of 26 specimens from UC patients, one specimen from a CD patient and one normal tissue specimen were positive for HSP40 [1].
  • Translation of mRNA for some expressed genes, including HSP70 and HSP40, was corroborated by Western blotting [28].
  • Similar results were obtained by overexpression of Hsp70 and Hsp40 in a separate cell culture model of HD [29].
  • This acetylation site mutant exhibits ligand-dependent 1C2 immunoreactivity, forms aggregates that co-localize with Hsp40, Hsp70, and the ubiquitin-protein isopeptide ligase (E3) ubiquitin ligase carboxyl terminus of Hsc70-interacting protein (CHIP), and inhibits proteasome function [30].


  1. Autoimmunity to heat shock protein 40 in ulcerative colitis. Sato, S., Oka, M., Noguchi, Y., Soda, H., Tsurutani, J., Nakamura, Y., Kitazaki, T., Mizuta, Y., Takeshima, F., Murase, K., Murata, I., Ohtsuka, K., Kohno, S. J. Int. Med. Res. (2004) [Pubmed]
  2. Viral pollution in the environment and in shellfish: human adenovirus detection by PCR as an index of human viruses. Pina, S., Puig, M., Lucena, F., Jofre, J., Girones, R. Appl. Environ. Microbiol. (1998) [Pubmed]
  3. Human origin of Bacteroides fragilis bacteriophages present in the environment. Tartera, C., Lucena, F., Jofre, J. Appl. Environ. Microbiol. (1989) [Pubmed]
  4. CSPalpha-deficiency causes massive and rapid photoreceptor degeneration. Schmitz, F., Tabares, L., Khimich, D., Strenzke, N., de la Villa-Polo, P., Castellano-Muñoz, M., Bulankina, A., Moser, T., Fernández-Chacón, R., Südhof, T.C. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  5. Gene expression profiling reveals a signaling role of glutathione in redox regulation. Fratelli, M., Goodwin, L.O., Ørom, U.A., Lombardi, S., Tonelli, R., Mengozzi, M., Ghezzi, P. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  6. The hsp70-associating protein Hap46 binds to DNA and stimulates transcription. Zeiner, M., Niyaz, Y., Gehring, U. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  7. A molecular pathogenesis for transcription factor associated poly-alanine tract expansions. Albrecht, A.N., Kornak, U., Böddrich, A., Süring, K., Robinson, P.N., Stiege, A.C., Lurz, R., Stricker, S., Wanker, E.E., Mundlos, S. Hum. Mol. Genet. (2004) [Pubmed]
  8. Dual function of membrane-bound heat shock protein 70 (Hsp70), Bag-4, and Hsp40: protection against radiation-induced effects and target structure for natural killer cells. Gehrmann, M., Marienhagen, J., Eichholtz-Wirth, H., Fritz, E., Ellwart, J., Jäättelä, M., Zilch, T., Multhoff, G. Cell Death Differ. (2005) [Pubmed]
  9. Increased expression of HDJ-2 (hsp40) in carotid artery atherosclerosis: a novel heat shock protein associated with luminal stenosis and plaque ulceration. Nguyen, T.Q., Jaramillo, A., Thompson, R.W., Dintzis, S., Oppat, W.F., Allen, B.T., Sicard, G.A., Mohanakumar, T. J. Vasc. Surg. (2001) [Pubmed]
  10. HSP-CBF is an NF-Y-dependent coactivator of the heat shock promoters CCAAT boxes. Imbriano, C., Bolognese, F., Gurtner, A., Piaggio, G., Mantovani, R. J. Biol. Chem. (2001) [Pubmed]
  11. Characterization of a processed pseudogene of human psiHSP40 on chromosome 2q32. Hata, M., Kagotani, K., Okumura, K., Seto, M., Ohtsuka, K. DNA Seq. (2001) [Pubmed]
  12. Description of a DNA amplification procedure for the detection of bacteriophages of Bacteroides fragilis HSP40 in environmental samples. Puig, M., Pina, S., Lucena, F., Jofre, J., Girones, R. J. Virol. Methods (2000) [Pubmed]
  13. HEDJ, an Hsp40 co-chaperone localized to the endoplasmic reticulum of human cells. Yu, M., Haslam, R.H., Haslam, D.B. J. Biol. Chem. (2000) [Pubmed]
  14. Aggresome formation in neuropathy models based on peripheral myelin protein 22 mutations. Ryan, M.C., Shooter, E.M., Notterpek, L. Neurobiol. Dis. (2002) [Pubmed]
  15. Molecular guardians for newborn proteins: ribosome-associated chaperones and their role in protein folding. Wegrzyn, R.D., Deuerling, E. Cell. Mol. Life Sci. (2005) [Pubmed]
  16. Translocation of constitutively expressed heat shock protein Hsc70 to synapse-enriched areas of the cerebral cortex after hyperthermic stress. Chen, S., Brown, I.R. J. Neurosci. Res. (2007) [Pubmed]
  17. Mitochondria-derived oxidative stress induces a heat shock protein response. Barrett, M.J., Alones, V., Wang, K.X., Phan, L., Swerdlow, R.H. J. Neurosci. Res. (2004) [Pubmed]
  18. A conserved motif is prerequisite for the interaction of NAC with ribosomal protein L23 and nascent chains. Wegrzyn, R.D., Hofmann, D., Merz, F., Nikolay, R., Rauch, T., Graf, C., Deuerling, E. J. Biol. Chem. (2006) [Pubmed]
  19. Hsp105alpha suppresses the aggregation of truncated androgen receptor with expanded CAG repeats and cell toxicity. Ishihara, K., Yamagishi, N., Saito, Y., Adachi, H., Kobayashi, Y., Sobue, G., Ohtsuka, K., Hatayama, T. J. Biol. Chem. (2003) [Pubmed]
  20. Anti-invasive gene expression profile of curcumin in lung adenocarcinoma based on a high throughput microarray analysis. Chen, H.W., Yu, S.L., Chen, J.J., Li, H.N., Lin, Y.C., Yao, P.L., Chou, H.Y., Chien, C.T., Chen, W.J., Lee, Y.T., Yang, P.C. Mol. Pharmacol. (2004) [Pubmed]
  21. Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein. Waza, M., Adachi, H., Katsuno, M., Minamiyama, M., Tanaka, F., Doyu, M., Sobue, G. J. Mol. Med. (2006) [Pubmed]
  22. Domain requirements of DnaJ-like (Hsp40) molecular chaperones in the activation of a steroid hormone receptor. Fliss, A.E., Rao, J., Melville, M.W., Cheetham, M.E., Caplan, A.J. J. Biol. Chem. (1999) [Pubmed]
  23. The J-domain of Hsp40 couples ATP hydrolysis to substrate capture in Hsp70. Wittung-Stafshede, P., Guidry, J., Horne, B.E., Landry, S.J. Biochemistry (2003) [Pubmed]
  24. Small molecule modulators of endogenous and co-chaperone-stimulated Hsp70 ATPase activity. Fewell, S.W., Smith, C.M., Lyon, M.A., Dumitrescu, T.P., Wipf, P., Day, B.W., Brodsky, J.L. J. Biol. Chem. (2004) [Pubmed]
  25. Spectrum and range of oxidative stress responses of human lens epithelial cells to H2O2 insult. Goswami, S., Sheets, N.L., Zavadil, J., Chauhan, B.K., Bottinger, E.P., Reddy, V.N., Kantorow, M., Cvekl, A. Invest. Ophthalmol. Vis. Sci. (2003) [Pubmed]
  26. Cdc48 can distinguish between native and non-native proteins in the absence of cofactors. Thoms, S. FEBS Lett. (2002) [Pubmed]
  27. Phenotypic anchoring of arsenic and cadmium toxicity in three hepatic-related cell systems reveals compound- and cell-specific selective up-regulation of stress protein expression: implications for fingerprint profiling of cytotoxicity. Gottschalg, E., Moore, N.E., Ryan, A.K., Travis, L.C., Waller, R.C., Pratt, S., Atmaca, M., Kind, C.N., Fry, J.R. Chem. Biol. Interact. (2006) [Pubmed]
  28. Gene expression profiling of the response to thermal injury in human cells. Dinh, H.K., Zhao, B., Schuschereba, S.T., Merrill, G., Bowman, P.D. Physiol. Genomics (2001) [Pubmed]
  29. Geldanamycin activates a heat shock response and inhibits huntingtin aggregation in a cell culture model of Huntington's disease. Sittler, A., Lurz, R., Lueder, G., Priller, J., Lehrach, H., Hayer-Hartl, M.K., Hartl, F.U., Wanker, E.E. Hum. Mol. Genet. (2001) [Pubmed]
  30. Androgen receptor acetylation site mutations cause trafficking defects, misfolding, and aggregation similar to expanded glutamine tracts. Thomas, M., Dadgar, N., Aphale, A., Harrell, J.M., Kunkel, R., Pratt, W.B., Lieberman, A.P. J. Biol. Chem. (2004) [Pubmed]
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