Gene Symbol: Mdj1p
Description: Mdj1p
Alias: Mdj1p
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Westermann B, Gaume B, Herrmann J, Neupert W, Schwarz E. Role of the mitochondrial DnaJ homolog Mdj1p as a chaperone for mitochondrially synthesized and imported proteins. Mol Cell Biol. 1996;16:7063-71 pubmed
    b>Mdj1p, a DnaJ homolog in the mitochondria of Saccharomyces cerevisiae, is involved in the folding of proteins in the mitochondrial matrix. In this capacity, Mdj1p cooperates with mitochondrial Hsp70 (mt-Hsp70)...
  2. D Silva P, Schilke B, Walter W, Andrew A, Craig E. J protein cochaperone of the mitochondrial inner membrane required for protein import into the mitochondrial matrix. Proc Natl Acad Sci U S A. 2003;100:13839-44 pubmed
    ..We propose that Pam18 is the J protein partner for Ssc1 at the import channel and is critical for Ssc1's function in protein import. ..
  3. Horst M, Oppliger W, Rospert S, Schönfeld H, Schatz G, Azem A. Sequential action of two hsp70 complexes during protein import into mitochondria. EMBO J. 1997;16:1842-9 pubmed
    ..A precursor protein entering the matrix interacts first with the import complex and then with the folding complex. A chaperone can thus function as part of two different complexes within the same organelle. ..
  4. Duchniewicz M, Germaniuk A, Westermann B, Neupert W, Schwarz E, Marszalek J. Dual role of the mitochondrial chaperone Mdj1p in inheritance of mitochondrial DNA in yeast. Mol Cell Biol. 1999;19:8201-10 pubmed
    b>Mdj1p, a homolog of the bacterial DnaJ chaperone protein, plays an essential role in the biogenesis of functional mitochondria in the yeast Saccharomyces cerevisiae...
  5. Pierrel F, Bestwick M, Cobine P, Khalimonchuk O, Cricco J, Winge D. Coa1 links the Mss51 post-translational function to Cox1 cofactor insertion in cytochrome c oxidase assembly. EMBO J. 2007;26:4335-46 pubmed
    ..coa1Delta cells also display a mitochondrial copper defect suggesting that Coa1 may have a direct link to copper metallation of CcO. ..
  6. Goswami A, Samaddar M, Sinha D, Purushotham J, D Silva P. Enhanced J-protein interaction and compromised protein stability of mtHsp70 variants lead to mitochondrial dysfunction in Parkinson's disease. Hum Mol Genet. 2012;21:3317-32 pubmed publisher
    ..Taken together, our findings provide evidence in favor of direct involvement of mtHsp70 as a susceptibility factor in PD. ..
  7. Prip Buus C, Westerman B, Schmitt M, Langer T, Neupert W, Schwarz E. Role of the mitochondrial DnaJ homologue, Mdj1p, in the prevention of heat-induced protein aggregation. FEBS Lett. 1996;380:142-6 pubmed 40 degrees Celsius transient increase of mt-Hsp70/luciferase complex was observed, which required functional Mdj1p and Mge1p, the mitochondrial homologues of DnaJ and GrpE...
  8. Westermann B, Neupert W. Mdj2p, a novel DnaJ homolog in the mitochondrial inner membrane of the yeast Saccharomyces cerevisiae. J Mol Biol. 1997;272:477-83 pubmed
    ..The mitochondrial DnaJ homolog, Mdj1p, has been shown to cooperate with the major mitochondrial Hsp70, mt-Hsp70...
  9. Kubo Y, Tsunehiro T, Nishikawa S, Nakai M, Ikeda E, Toh e A, et al. Two distinct mechanisms operate in the reactivation of heat-denatured proteins by the mitochondrial Hsp70/Mdj1p/Yge1p chaperone system. J Mol Biol. 1999;286:447-64 pubmed
    The yeast mitochondrial Hsp70, Ssc1p, functions as a molecular chaperone with its partner proteins, Mdj1p (DnaJ homologue) and Yge1p (GrpE homologue)...

More Information


  1. Burri L, Vascotto K, Fredersdorf S, Tiedt R, Hall M, Lithgow T. Zim17, a novel zinc finger protein essential for protein import into mitochondria. J Biol Chem. 2004;279:50243-9 pubmed
    ..We propose that Zim17 functions as an example of a "fractured" J-protein, where a protein like Zim17 contributes a zinc finger domain to Type III J-proteins, in toto providing for substrate loading onto Hsp70. ..
  2. Fontanesi F, SOTO I, Horn D, Barrientos A. Mss51 and Ssc1 facilitate translational regulation of cytochrome c oxidase biogenesis. Mol Cell Biol. 2010;30:245-59 pubmed publisher
    ..We conclude that Ssc1, by interacting with Mss51 and Mss51-containing complexes, plays a critical role in Cox1 biogenesis, COX assembly, and the translational regulation of these processes. ..
  3. Becker D, Krayl M, Strub A, Li Y, Mayer M, Voos W. Impaired interdomain communication in mitochondrial Hsp70 results in the loss of inward-directed translocation force. J Biol Chem. 2009;284:2934-46 pubmed publisher
    ..We conclude that even a partial disruption of the interdomain communication in the mtHsp70 chaperone results in an almost complete breakdown of its translocation-driving properties. ..
  4. Jackson T, Kwon E, Chachulska A, Hyman L. Novel roles for elongin C in yeast. Biochim Biophys Acta. 2000;1491:161-76 pubmed
    ..This suggests a novel role for Elongin C and provides insights into additional potential functions in mammalian cells. ..
  5. Mapa K, Sikor M, Kudryavtsev V, Waegemann K, Kalinin S, Seidel C, et al. The conformational dynamics of the mitochondrial Hsp70 chaperone. Mol Cell. 2010;38:89-100 pubmed publisher
  6. Pierrel F, Khalimonchuk O, Cobine P, Bestwick M, Winge D. Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of Cox1. Mol Cell Biol. 2008;28:4927-39 pubmed publisher
    ..The matrix-localized Coa2 protein may aid in stabilizing an early Cox1 intermediate containing the nuclear subunits Cox5a and Cox6. ..
  7. Wagner I, Arlt H, van Dyck L, Langer T, Neupert W. Molecular chaperones cooperate with PIM1 protease in the degradation of misfolded proteins in mitochondria. EMBO J. 1994;13:5135-45 pubmed
    ..mitochondrial matrix is mediated by the PIM1 protease and depends on the molecular chaperone proteins mt-hsp70 and Mdj1p. Chaperone function is essential to maintain misfolded proteins in a soluble state, a prerequisite for their ..
  8. Rowley N, Prip Buus C, Westermann B, Brown C, Schwarz E, Barrell B, et al. Mdj1p, a novel chaperone of the DnaJ family, is involved in mitochondrial biogenesis and protein folding. Cell. 1994;77:249-59 pubmed
    b>Mdj1p, a novel member of the DnaJ family, is a heat shock protein that is associated with the inner membrane of mitochondria of Saccharomyces cerevisiae...