Genomes and Genes
Experts and Doctors on mitochondria in Munich, Bavaria, Germany
Locale: Munich, Bavaria, Germany
- Dirsch V, Müller I, Eichhorst S, Pettit G, Kamano Y, Inoue M, et al. Cephalostatin 1 selectively triggers the release of Smac/DIABLO and subsequent apoptosis that is characterized by an increased density of the mitochondrial matrix. Cancer Res. 2003;63:8869-76 pubmed..This new mitochondrial pathway is accompanied by marked structural changes of mitochondria as shown by transmission electron microscopy. ..
- Shakibaei M, Buhrmann C, Mobasheri A. Anti-inflammatory and anti-catabolic effects of TENDOACTIVE® on human tenocytes in vitro. Histol Histopathol. 2011;26:1173-85 pubmed publisher..These results indicate that Tendoactive® has nutraceutical potential as an anti-inflammatory agent for treating tendinopathy through suppression of NF-?B mediated IL-1? catabolic signalling pathways in tenocytes. ..
- Vidali S, Knuever J, Lerchner J, Giesen M, Biro T, Klinger M, et al. Hypothalamic-pituitary-thyroid axis hormones stimulate mitochondrial function and biogenesis in human hair follicles. J Invest Dermatol. 2014;134:33-42 pubmed publisher..The neuroendocrine control of mitochondrial biology in a complex human mini-organ revealed here may be therapeutically exploitable. ..
- Huang S, Odoy S, Klingenberg M. Chimers of two fused ADP/ATP carrier monomers indicate a single channel for ADP/ATP transport. Arch Biochem Biophys. 2001;394:67-75 pubmed..These results favor a single binding center gated pore model [Klingenberg, M. (1991) in A Study of Enzymes, Vol. 2: pp. 367-388] in which two AAC subunits cooperate for a coordinated ADP/ATP exchange through a single channel. ..
- Exner N, Treske B, Paquet D, Holmström K, Schiesling C, Gispert S, et al. Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin. J Neurosci. 2007;27:12413-8 pubmed..Our results may therefore suggest that PINK1 deficiency in humans results in mitochondrial abnormalities associated with cellular stress, a pathological phenotype, which can be ameliorated by enhanced expression of parkin. ..
- Fritsch R, Schneider G, Saur D, Scheibel M, Schmid R. Translational repression of MCL-1 couples stress-induced eIF2 alpha phosphorylation to mitochondrial apoptosis initiation. J Biol Chem. 2007;282:22551-62 pubmed..Down-regulation of MCL-1 enables but not enforces apoptosis initiation in stressed cells. ..
- Weber A, Paschen S, Heger K, Wilfling F, Frankenberg T, Bauerschmitt H, et al. BimS-induced apoptosis requires mitochondrial localization but not interaction with anti-apoptotic Bcl-2 proteins. J Cell Biol. 2007;177:625-36 pubmed..Thus, cell death induction by a BH3-only protein can occur through a process that is independent of anti-apoptotic Bcl-2 proteins but requires mitochondrial targeting. ..
- Court D, Nargang F, Steiner H, Hodges R, Neupert W, Lill R. Role of the intermembrane-space domain of the preprotein receptor Tom22 in protein import into mitochondria. Mol Cell Biol. 1996;16:4035-42 pubmed..Rather, the IMS domain of Tom22 appears to slightly enhance the efficiency of the transfer of these preproteins to the import machinery of the inner membrane. ..
- Stoetzer O, Pogrebniak A, Pelka Fleischer R, Hasmann M, Hiddemann W, Nuessler V. Modulation of apoptosis by mitochondrial uncouplers: apoptosis-delaying features despite intrinsic cytotoxicity. Biochem Pharmacol. 2002;63:471-83 pubmed..However, when used in combination with serine/threonine protein kinase inhibitors FCCP can even prevent apoptosis. ..
- Jaksch M, Paret C, Stucka R, Horn N, Müller Höcker J, Horvath R, et al. Cytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts. Hum Mol Genet. 2001;10:3025-35 pubmed..Whatever the mechanism, this result suggests a possible therapy for the early treatment of this fatal infantile disease. ..
- Moro F, Okamoto K, Donzeau M, Neupert W, Brunner M. Mitochondrial protein import: molecular basis of the ATP-dependent interaction of MtHsp70 with Tim44. J Biol Chem. 2002;277:6874-80 pubmed
- Müller T, Lee S, Jastroch M, Kabra D, Stemmer K, Aichler M, et al. p62 links β-adrenergic input to mitochondrial function and thermogenesis. J Clin Invest. 2013;123:469-78 pubmed publisher..Together, our data identify p62 as a novel regulator of mitochondrial function and brown fat thermogenesis. ..
- Betz M, Bielohuby M, Mauracher B, Abplanalp W, Muller H, Pieper K, et al. Isoenergetic feeding of low carbohydrate-high fat diets does not increase brown adipose tissue thermogenic capacity in rats. PLoS ONE. 2012;7:e38997 pubmed publisher..We studied brown adipose tissue (BAT) morphology and function following exposure to different LC-HF diets...
- Duvezin Caubet S, Koppen M, Wagener J, Zick M, Israel L, Bernacchia A, et al. OPA1 processing reconstituted in yeast depends on the subunit composition of the m-AAA protease in mitochondria. Mol Biol Cell. 2007;18:3582-90 pubmed
- Koch J, Feichtinger R, Freisinger P, Pies M, SchrÃ¶dl F, Iuso A, et al. Disturbed mitochondrial and peroxisomal dynamics due to loss of MFF causes Leigh-like encephalopathy, optic atrophy and peripheral neuropathy. J Med Genet. 2016;53:270-8 pubmed publisher..We suggest that, even if laboratory findings are not indicative of mitochondrial or peroxisomal dysfunction, the co-occurrence of optic and/or peripheral neuropathy with seizures warrants genetic testing for MFF mutations. ..
- Reiter V, Matschkal D, Wagner M, Globisch D, Kneuttinger A, Muller M, et al. The CDK5 repressor CDK5RAP1 is a methylthiotransferase acting on nuclear and mitochondrial RNA. Nucleic Acids Res. 2012;40:6235-40 pubmed publisher..Instead, CDK5RAP1 introduces the modification also into nuclear RNA species establishing a link between postsynthetic kinase-based protein modification and postsynthetic RNA modification. ..
- Hell K, Herrmann J, Pratje E, Neupert W, Stuart R. Oxa1p mediates the export of the N- and C-termini of pCoxII from the mitochondrial matrix to the intermembrane space. FEBS Lett. 1997;418:367-70 pubmed..In conclusion, Oxa1p mediates the export of the N- and C-termini of the mitochondrially encoded subunit II of cytochrome oxidase from the matrix to the intermembrane space. ..
- Habarou F, Hamel Y, Haack T, Feichtinger R, Lebigot E, Marquardt I, et al. Biallelic Mutations in LIPT2 Cause a Mitochondrial Lipoylation Defect Associated with Severe Neonatal Encephalopathy. Am J Hum Genet. 2017;101:283-290 pubmed publisher..Lipoic acid supplementation did not improve clinical condition nor activities of PDHc, ?-KGDHc, or leucine metabolism in fibroblasts and was ineffective in yeast deleted for the orthologous LIP2. ..
- Arnold I, Bauer M, Brunner M, Neupert W, Stuart R. Yeast mitochondrial F1F0-ATPase: the novel subunit e is identical to Tim11. FEBS Lett. 1997;411:195-200 pubmed..Subunit e had been previously identified as Tim11 and was proposed to be involved in the process of sorting of proteins to the mitochondrial inner membrane. ..
- van Dyck L, Dembowski M, Neupert W, Langer T. Mcx1p, a ClpX homologue in mitochondria of Saccharomyces cerevisiae. FEBS Lett. 1998;438:250-4 pubmed..A homologue of E. coli ClpP protease was not identified when screening the yeast genome. We therefore propose that Mcx1p represents a novel molecular chaperone of mitochondria with non-proteolytic function. ..
- Kozany C, Mokranjac D, Sichting M, Neupert W, Hell K. The J domain-related cochaperone Tim16 is a constituent of the mitochondrial TIM23 preprotein translocase. Nat Struct Mol Biol. 2004;11:234-41 pubmed..In conclusion, Tim16 is a constituent of the TIM23 preprotein translocase, where it exerts crucial functions in the import motor. ..
- Steglich G, Neupert W, Langer T. Prohibitins regulate membrane protein degradation by the m-AAA protease in mitochondria. Mol Cell Biol. 1999;19:3435-42 pubmed..These results functionally link members of two conserved protein families in eukaryotes to the degradation of membrane proteins in mitochondria. ..
- Funes S, Hasona A, Bauerschmitt H, Grubbauer C, Kauff F, Collins R, et al. Independent gene duplications of the YidC/Oxa/Alb3 family enabled a specialized cotranslational function. Proc Natl Acad Sci U S A. 2009;106:6656-61 pubmed publisher..Our results are consistent with a gene-duplication event in gram-positive bacteria that enabled the specialization of a YidC isoform that mediates cotranslational activity independent of an SRP pathway. ..
- Stadler S, Polanetz R, Meier S, Mayerhofer P, Herrmann J, Anslinger K, et al. Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase. Biochem Biophys Res Commun. 2005;334:939-46 pubmed..Structural requirements for mitochondrial import were defined by site-directed mutagenesis. Our studies provide the prerequisite to understand the impact of specific mutations on the clinical phenotype of MCC deficiency. ..
- Schusdziarra C, Blamowska M, Azem A, Hell K. Methylation-controlled J-protein MCJ acts in the import of proteins into human mitochondria. Hum Mol Genet. 2013;22:1348-57 pubmed publisher..In summary, our results show that MCJ functions as J co-chaperone of the human TIM23 pre-protein translocase, suggesting a link between mitochondrial pre-protein import and tumorigenesis. ..
- Bender A, Desplats P, Spencer B, Rockenstein E, Adame A, Elstner M, et al. TOM40 mediates mitochondrial dysfunction induced by ?-synuclein accumulation in Parkinson's disease. PLoS ONE. 2013;8:e62277 pubmed publisher..Our results suggest that alterations in the mitochondrial protein transport machinery might contribute to mitochondrial impairment in ?-Synucleinopathies. ..
- Popov Celeketic D, Waegemann K, Mapa K, Neupert W, Mokranjac D. Role of the import motor in insertion of transmembrane segments by the mitochondrial TIM23 complex. EMBO Rep. 2011;12:542-8 pubmed publisher..Furthermore, our results suggest a role for the import motor in lateral insertion. Thus, the import motor is involved in ATP-dependent translocation and ATP-independent lateral insertion. ..
- Hell K, Herrmann J, Pratje E, Neupert W, Stuart R. Oxa1p, an essential component of the N-tail protein export machinery in mitochondria. Proc Natl Acad Sci U S A. 1998;95:2250-5 pubmed..Thus, Oxa1p represents a component of a general export machinery of the mitochondrial inner membrane. ..
- Haack T, Kopajtich R, Freisinger P, Wieland T, Rorbach J, Nicholls T, et al. ELAC2 mutations cause a mitochondrial RNA processing defect associated with hypertrophic cardiomyopathy. Am J Hum Genet. 2013;93:211-23 pubmed publisher..Complementation experiments in mutant cell lines restored RNA processing and a yeast model provided additional evidence for the disease-causal role of defective ELAC2, thereby linking mtRNA processing to human disease. ..
- Schreiner B, Westerburg H, Forne I, Imhof A, Neupert W, Mokranjac D. Role of the AAA protease Yme1 in folding of proteins in the intermembrane space of mitochondria. Mol Biol Cell. 2012;23:4335-46 pubmed publisher..These findings explain the pleiotropic effects of deletion of YME1 and suggest an important role for Yme1 as a folding assistant, in addition to its proteolytic function, in the protein homeostasis of mitochondria. ..
- Büsch F, Mobasheri A, Shayan P, Lueders C, Stahlmann R, Shakibaei M. Resveratrol modulates interleukin-1?-induced phosphatidylinositol 3-kinase and nuclear factor ?B signaling pathways in human tenocytes. J Biol Chem. 2012;287:38050-63 pubmed publisher..Overall, these results demonstrate that activated Sirt-1 plays an essential role in the anti-inflammatory effects of resveratrol and this may be mediated at least in part through inhibition/deacetylation of PI3K and NF-?B. ..
- Popov Celeketic D, Mapa K, Neupert W, Mokranjac D. Active remodelling of the TIM23 complex during translocation of preproteins into mitochondria. EMBO J. 2008;27:1469-80 pubmed publisher..Our data demonstrate that the TIM23 complex acts as a single structural and functional entity that is actively remodelled to sort preproteins into different mitochondrial subcompartments. ..
- Waizenegger T, Habib S, Lech M, Mokranjac D, Paschen S, Hell K, et al. Tob38, a novel essential component in the biogenesis of beta-barrel proteins of mitochondria. EMBO Rep. 2004;5:704-9 pubmed..We conclude that Tob38 has a crucial function in the biogenesis of beta-barrel proteins of mitochondria. ..
- García Cáceres C, Quarta C, Varela L, Gao Y, Gruber T, Legutko B, et al. Astrocytic Insulin Signaling Couples Brain Glucose Uptake with Nutrient Availability. Cell. 2016;166:867-880 pubmed publisher..We conclude that insulin signaling in hypothalamic astrocytes co-controls CNS glucose sensing and systemic glucose metabolism via regulation of glucose uptake across the BBB. ..
- Preuss M, Leonhard K, Hell K, Stuart R, Neupert W, Herrmann J. Mba1, a novel component of the mitochondrial protein export machinery of the yeast Saccharomyces cerevisiae. J Cell Biol. 2001;153:1085-96 pubmed..We conclude that Mba1 is part of the mitochondrial protein export machinery and represents the first component of a novel Oxa1-independent insertion pathway into the mitochondrial inner membrane. ..
- Hendrich L, Pons J, Ribera I, Balke M. Mitochondrial cox1 sequence data reliably uncover patterns of insect diversity but suffer from high lineage-idiosyncratic error rates. PLoS ONE. 2010;5:e14448 pubmed publisher..However, error rates can be high in individual lineages. ..
- Horstkotte J, Perisic T, Schneider M, Lange P, Schroeder M, Kiermayer C, et al. Mitochondrial thioredoxin reductase is essential for early postischemic myocardial protection. Circulation. 2011;124:2892-902 pubmed publisher..The efficacy of cyclosporin A in cardiac Txnrd2 deficiency may indicate a role for Txnrd2 in reducing mitochondrial reactive oxygen species, thereby preventing opening of the mitochondrial permeability transition pore. ..
- Bauer M, Rothbauer U, Mühlenbein N, Smith R, Gerbitz K, Neupert W, et al. The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom. FEBS Lett. 1999;464:41-7 pubmed..We report the identification, chromosomal localization and expressional analysis of six human family members which represent further candidate genes for neurodegenerative diseases. ..
- Hall A, Lächelt U, Bartek J, Wagner E, Moghimi S. Polyplex Evolution: Understanding Biology, Optimizing Performance. Mol Ther. 2017;25:1476-1490 pubmed publisher..These perspectives are intended to lay the foundation for an improved understanding of interlinked mechanisms controlling transfection and toxicity and their translation for improved engineering of polycation-based transfectants. ..
- Rabl R, Soubannier V, Scholz R, Vogel F, Mendl N, Vasiljev Neumeyer A, et al. Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Su e/g. J Cell Biol. 2009;185:1047-63 pubmed publisher..We propose a model in which the antagonism between Fcj1 and Su e/g locally modulates the F(1)F(O) oligomeric state, thereby controlling membrane curvature of cristae to generate CJs and cristae tips. ..
- Yuan D, Huang S, Berger E, Liu L, Gross N, Heinzmann F, et al. Kupffer Cell-Derived Tnf Triggers Cholangiocellular Tumorigenesis through JNK due to Chronic Mitochondrial Dysfunction and ROS. Cancer Cell. 2017;31:771-789.e6 pubmed publisher..Thus, Kupffer cell-derived Tnf favors cholangiocellular proliferation/differentiation and carcinogenesis. Targeting the ROS/Tnf/JNK axis may provide opportunities for ICC therapy. ..
- Waizenegger T, Schmitt S, Zivkovic J, Neupert W, Rapaport D. Mim1, a protein required for the assembly of the TOM complex of mitochondria. EMBO Rep. 2005;6:57-62 pubmed..Mim1 is a constituent of neither the TOM complex nor the TOB complex; rather, it seems to be a subunit of another, as yet unidentified, complex. We conclude that Mim1 has a vital and specific function in the assembly of the TOM complex. ..
- Habib S, Waizenegger T, Niewienda A, Paschen S, Neupert W, Rapaport D. The N-terminal domain of Tob55 has a receptor-like function in the biogenesis of mitochondrial beta-barrel proteins. J Cell Biol. 2007;176:77-88 pubmed..This recognition may contribute to the coupling of the translocation of beta-barrel precursors across the TOM complex to their interaction with the TOB complex. ..
- Zall H, Weber A, Besch R, Zantl N, Hacker G. Chemotherapeutic drugs sensitize human renal cell carcinoma cells to ABT-737 by a mechanism involving the Noxa-dependent inactivation of Mcl-1 or A1. Mol Cancer. 2010;9:164 pubmed publisher..Strikingly, A1 emerged in RCC cell lines as a protein of similar importance as the well-established Mcl-1 in protection against apoptosis in these cells. ..
- Brüning A, Burger P, Vogel M, Gingelmaier A, Friese K, Burges A. Nelfinavir induces mitochondria protection by ERK1/2-mediated mcl-1 stabilization that can be overcome by sorafenib. Invest New Drugs. 2010;28:535-42 pubmed publisher..Thus, a combination of these two investigational anti-cancer drugs could be of interest especially because of their unique mechanism of apoptosis induction even in otherwise chemo-resistant human cancer cells. ..
- Lutz A, Exner N, Fett M, Schlehe J, Kloos K, Lämmermann K, et al. Loss of parkin or PINK1 function increases Drp1-dependent mitochondrial fragmentation. J Biol Chem. 2009;284:22938-51 pubmed publisher..We propose that the discrepant findings in adult flies can be explained by the time of phenotype analysis and suggest that in mammals different strategies may have evolved to cope with dysfunctional mitochondria. ..
- Marinkovic P, Reuter M, Brill M, Godinho L, Kerschensteiner M, Misgeld T. Axonal transport deficits and degeneration can evolve independently in mouse models of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A. 2012;109:4296-301 pubmed publisher..These data suggest that disturbances of organelle transport are not a necessary step in the emergence of motor neuron degeneration...
- Sibbing D, Pfeufer A, Perisic T, Mannes A, Fritz Wolf K, Unwin S, et al. Mutations in the mitochondrial thioredoxin reductase gene TXNRD2 cause dilated cardiomyopathy. Eur Heart J. 2011;32:1121-33 pubmed publisher..For the first time, we describe mutations in DCM patients in a gene involved in the regulation of cellular redox state. TXNRD2 mutations may explain a fraction of human DCM disease burden. ..
- Schleiff E, Soll J. Membrane protein insertion: mixing eukaryotic and prokaryotic concepts. EMBO Rep. 2005;6:1023-7 pubmed..This review focuses on recent advances in the elucidation of the evolutionarily conserved concepts that underlie the translocation and insertion of beta-barrel membrane proteins. ..
- Bauerschmitt H, Funes S, Herrmann J. The membrane-bound GTPase Guf1 promotes mitochondrial protein synthesis under suboptimal conditions. J Biol Chem. 2008;283:17139-46 pubmed publisher..These observations show a critical role for Guf1 in vivo. The observed defects in Guf1-deficient mitochondria are consistent with a function of Guf1 as a fidelity factor of mitochondrial protein synthesis. ..
- Landshamer S, Hoehn M, Barth N, Duvezin Caubet S, Schwake G, Tobaben S, et al. Bid-induced release of AIF from mitochondria causes immediate neuronal cell death. Cell Death Differ. 2008;15:1553-63 pubmed publisher..In conclusion, Bid-mediated mitochondrial release of AIF followed by rapid nuclear translocation is a major mechanism of glutamate-induced neuronal death. ..
- Hartig M, Iuso A, Haack T, Kmiec T, Jurkiewicz E, Heim K, et al. Absence of an orphan mitochondrial protein, c19orf12, causes a distinct clinical subtype of neurodegeneration with brain iron accumulation. Am J Hum Genet. 2011;89:543-50 pubmed publisher..The mitochondrial localization together with the immunohistopathological findings suggests a pathomechanistic overlap with common forms of neurodegenerative disorders. ..
- Dusi S, Valletta L, Haack T, Tsuchiya Y, Venco P, Pasqualato S, et al. Exome sequence reveals mutations in CoA synthase as a cause of neurodegeneration with brain iron accumulation. Am J Hum Genet. 2014;94:11-22 pubmed publisher..This is the second inborn error of coenzyme A biosynthesis to be implicated in NBIA. ..
- Kamp F, Exner N, Lutz A, Wender N, Hegermann J, Brunner B, et al. Inhibition of mitochondrial fusion by ?-synuclein is rescued by PINK1, Parkin and DJ-1. EMBO J. 2010;29:3571-89 pubmed publisher..Finally, mitochondrial fragmentation induced by expression of ?S is rescued by coexpression of PINK1, parkin or DJ-1 but not the PD-associated mutations PINK1 G309D and parkin ?1-79 or by DJ-1 C106A. ..