Experts and Doctors on mitochondrial proteins in Austria


Locale: Austria
Topic: mitochondrial proteins

Top Publications

  1. Beretta M, Sottler A, Schmidt K, Mayer B, Gorren A. Partially irreversible inactivation of mitochondrial aldehyde dehydrogenase by nitroglycerin. J Biol Chem. 2008;283:30735-44 pubmed publisher
    ..These observations are consistent with a significant role for irreversible inactivation of ALDH2 in the development of nitrate tolerance. ..
  2. Di Bartolomeo F, Doan K, Athenstaedt K, Becker T, Daum G. Involvement of a putative substrate binding site in the biogenesis and assembly of phosphatidylserine decarboxylase 1 from Saccharomyces cerevisiae. Biochim Biophys Acta Mol Cell Biol Lipids. 2017;1862:716-725 pubmed publisher
    ..Our data show that mutations in this motif affect processing and stability of Psd1p, and consequently the enzyme's activity. Thus, we conclude that this consensus motif is essential for structural integrity and processing of Psd1p. ..
  3. Kolisek M, Zsurka G, Samaj J, Weghuber J, Schweyen R, Schweigel M. Mrs2p is an essential component of the major electrophoretic Mg2+ influx system in mitochondria. EMBO J. 2003;22:1235-44 pubmed
    ..Taken together, these findings characterize Mrs2p as the first molecularly identified metal ion channel protein in the inner mitochondrial membrane. ..
  4. Dungel P, Haindl S, Behling T, Mayer B, Redl H, Kozlov A. Neither nitrite nor nitric oxide mediate toxic effects of nitroglycerin on mitochondria. J Biochem Mol Toxicol. 2011;25:297-302 pubmed publisher
    ..In summary, our data suggest that although NG induces vasodilatation via NO release, it causes mitochondrial dysfunction via an NO-independent pathway...
  5. Pircher H, Straganz G, Ehehalt D, Morrow G, Tanguay R, Jansen Durr P. Identification of human fumarylacetoacetate hydrolase domain-containing protein 1 (FAHD1) as a novel mitochondrial acylpyruvase. J Biol Chem. 2011;286:36500-8 pubmed publisher
    ..FAHD1 was also found in several human cell lines, where it localized to mitochondria. In summary, the current work identified mammalian FAHD1 as a novel mitochondrial enzyme with acylpyruvate hydrolase activity. ..
  6. Nebauer R, Schuiki I, Kulterer B, Trajanoski Z, Daum G. The phosphatidylethanolamine level of yeast mitochondria is affected by the mitochondrial components Oxa1p and Yme1p. FEBS J. 2007;274:6180-90 pubmed
    ..In summary, our results demonstrate a link between the mitochondrial protein import machinery, assembly and stability of Psd1p, and phosphatidylethanolamine homeostasis in yeast mitochondria. ..
  7. Weghuber J, Dieterich F, Froschauer E, Svidova S, Schweyen R. Mutational analysis of functional domains in Mrs2p, the mitochondrial Mg2+ channel protein of Saccharomyces cerevisiae. FEBS J. 2006;273:1198-209 pubmed
    ..Data presented support the notion that effects of mutations in Mrs2p on group II intron splicing are a consequence of changes in steady-state mitochondrial Mg2+ concentrations. ..
  8. Oberkofler H, Klein K, Felder T, Krempler F, Patsch W. Role of peroxisome proliferator-activated receptor-gamma coactivator-1alpha in the transcriptional regulation of the human uncoupling protein 2 gene in INS-1E cells. Endocrinology. 2006;147:966-76 pubmed
  9. Nowikovsky K, Pozzan T, Rizzuto R, Scorrano L, Bernardi P. Perspectives on: SGP symposium on mitochondrial physiology and medicine: the pathophysiology of LETM1. J Gen Physiol. 2012;139:445-54 pubmed publisher

More Information


  1. Hell Pourmojib M, Neuner P, Fischer H, Rezaie S, Kindås Mügge I, Knobler R, et al. Differential expression of a novel gene in response to hsp27 and cell differentiation in human keratinocytes. J Invest Dermatol. 2002;119:154-9 pubmed
    ..These results not only demonstrate for the first time that overexpression of hsp27 by gene transfer is associated with regulation of gene expression but also reveal a novel differentiation-associated gene in human keratinocytes. ..
  2. Oberegger H, Schoeser M, Zadra I, Abt B, Haas H. SREA is involved in regulation of siderophore biosynthesis, utilization and uptake in Aspergillus nidulans. Mol Microbiol. 2001;41:1077-89 pubmed
    ..amcA encodes a putative mitochondrial carrier for the siderophore precursor ornithine, indicating cross-regulation of siderophore and ornithine metabolism...
  3. Piskacek M, Zotova L, Zsurka G, Schweyen R. Conditional knockdown of hMRS2 results in loss of mitochondrial Mg(2+) uptake and cell death. J Cell Mol Med. 2009;13:693-700 pubmed publisher
    ..We conclude that hMrs2 is the major transport protein for Mg (+) uptake into mitochondria and that expression of hMrs2 is essential for the maintenance of respiratory complex I and cell viability. ..
  4. Mayr J, Haack T, Graf E, Zimmermann F, Wieland T, Haberberger B, et al. Lack of the mitochondrial protein acylglycerol kinase causes Sengers syndrome. Am J Hum Genet. 2012;90:314-20 pubmed publisher
  5. Horvath S, Böttinger L, Vögtle F, Wiedemann N, Meisinger C, Becker T, et al. Processing and topology of the yeast mitochondrial phosphatidylserine decarboxylase 1. J Biol Chem. 2012;287:36744-55 pubmed publisher
    ..In summary, membrane integration of Psd1 is crucial for its functionality and for maintenance of mitochondrial lipid homeostasis. ..
  6. Neubauer R, Neubauer A, Wölkart G, Schwarzenegger C, Lang B, Schmidt K, et al. Potent inhibition of aldehyde dehydrogenase-2 by diphenyleneiodonium: focus on nitroglycerin bioactivation. Mol Pharmacol. 2013;84:407-14 pubmed publisher
    ..In addition, the data provide new evidence for the essential role of ALDH2 in GTN bioactivation and may have implications to other fields of ALDH2 research, such as hepatic ethanol metabolism and cardiac ischemia/reperfusion injury. ..
  7. Froschauer E, Rietzschel N, Hassler M, Binder M, Schweyen R, Lill R, et al. The mitochondrial carrier Rim2 co-imports pyrimidine nucleotides and iron. Biochem J. 2013;455:57-65 pubmed publisher
  8. Zotova L, Aleschko M, Sponder G, Baumgartner R, Reipert S, Prinz M, et al. Novel components of an active mitochondrial K(+)/H(+) exchange. J Biol Chem. 2010;285:14399-414 pubmed publisher
    ..Based on these results, we propose that Ydl183p and the Mdm38p homologues Mrs7p, LETM1, and HCCR-1 are involved in the formation of an active KHE system. ..
  9. Froschauer E, Schweyen R, Wiesenberger G. The yeast mitochondrial carrier proteins Mrs3p/Mrs4p mediate iron transport across the inner mitochondrial membrane. Biochim Biophys Acta. 2009;1788:1044-50 pubmed publisher
    ..We conclude that the carrier proteins Mrs3p and Mrs4p transport Fe(2+) across the inner mitochondrial membrane. Their activity is dependent on the pH gradient and it is stimulated by iron shortage. ..
  10. Kirchner L, Chen W, Afjehi Sadat L, Viidik A, Skalicky M, Hoger H, et al. Hippocampal metabolic proteins are modulated in voluntary and treadmill exercise rats. Exp Neurol. 2008;212:145-51 pubmed publisher
  11. Wiesenberger G, Link T, von Ahsen U, Waldherr M, Schweyen R. MRS3 and MRS4, two suppressors of mtRNA splicing defects in yeast, are new members of the mitochondrial carrier family. J Mol Biol. 1991;217:23-37 pubmed
    ..The findings are discussed in relation to possible functions of the MRS proteins in mitochondrial solute translocation and RNA splicing. ..
  12. Kiechl S, Horvath R, Luoma P, Kiechl Kohlendorfer U, Wallacher Scholz B, Stucka R, et al. Two families with autosomal dominant progressive external ophthalmoplegia. J Neurol Neurosurg Psychiatry. 2004;75:1125-8 pubmed
    ..We report here the clinical and genetic features of two new families with autosomal dominant progressive external ophthalmoplegia (adPEO)...
  13. Doepper S, Kacani L, Falkensammer B, Dierich M, Stoiber H. Complement receptors in HIV infection. Curr Mol Med. 2002;2:703-11 pubmed
  14. Steinberger P, Szekeres A, Wille S, Stockl J, Selenko N, Prager E, et al. Identification of human CD93 as the phagocytic C1q receptor (C1qRp) by expression cloning. J Leukoc Biol. 2002;71:133-40 pubmed
    ..Furthermore, we show that immature dendritic cells (DC) express CD93/C1qRp, and mature DC, known to have reduced capacity for antigen uptake and to have lost the ability to phagocytose, show weak-to-negative CD93/C1qRp expression. ..
  15. Gregan J, Bui D, Pillich R, Fink M, Zsurka G, Schweyen R. The mitochondrial inner membrane protein Lpe10p, a homologue of Mrs2p, is essential for magnesium homeostasis and group II intron splicing in yeast. Mol Gen Genet. 2001;264:773-81 pubmed
    ..However, they cannot easily substitute for each other. Their roles in magnesium homeostasis and, possibly as a secondary effect, in RNA splicing are discussed. ..
  16. Bui D, Gregan J, Jarosch E, Ragnini A, Schweyen R. The bacterial magnesium transporter CorA can functionally substitute for its putative homologue Mrs2p in the yeast inner mitochondrial membrane. J Biol Chem. 1999;274:20438-43 pubmed
    ..Mutants lacking or overproducing Mrs2p exhibit phenotypes consistent with the involvement of Mrs2p in mitochondrial Mg2+ homeostasis. ..
  17. Oberkofler H, Hafner M, Felder T, Krempler F, Patsch W. Transcriptional co-activator peroxisome proliferator-activated receptor (PPAR)gamma co-activator-1beta is involved in the regulation of glucose-stimulated insulin secretion in INS-1E cells. J Mol Med (Berl). 2009;87:299-306 pubmed publisher
    ..Our results support a role of PGC-1beta in the regulation of insulin secretion via upregulation of UCP2 and granuphilin gene expression. ..
  18. Esterbauer H, Schneitler C, Oberkofler H, Ebenbichler C, Paulweber B, Sandhofer F, et al. A common polymorphism in the promoter of UCP2 is associated with decreased risk of obesity in middle-aged humans. Nat Genet. 2001;28:178-83 pubmed
    ..Despite its modest effect, but because of its high frequency (approximately 63%), the more-common risk allele conferred a relatively large population-attributable risk accounting for 15% of the obesity in the population studied. ..