Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms

Gieffers C, Korioth F, Heimann P, Ungermann C, Frey J (1997)

Zeitschriftenaufsatz | Veröffentlicht | Englisch
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Gieffers, C; Korioth, F; Heimann, PeterUniBi; Ungermann, C; Frey, JürgenUniBi
Abstract / Bemerkung
Mitofilin, also known as heart muscle protein, is a recently identified mitochondrial protein. We have isolated two human cDNAs that encode different isoforms of mitofilin. Using reverse PCR, we provide evidence that both isoforms are derived by alternative splicing and encode two proteins of 88 and 90 kDa that are detected in immunoblot analyses with mitofilin-specific antibodies, Immunofluorescence microscopy, fractionating of human osteosarcoma cells, and protease protection experiments with isolated mitochondria and mitoplasts indicate that mitofilin is an integral membrane protein of the inner mitochondrial membrane. S-35-labeled mitofilin is transported into isolated yeast mitochondria in a reaction that depends on the membrane potential across the inner mitochondrial membrane (Delta Psi). During mitochondrial in vitro import, mitofilin is proteolytically processed to the mature protein that is also detected in cellular fractions, indicating that the amino-terminal leader sequence is removed, Sequence analysis and our re suits suggest that mitofilin is anchored in the inner mitochondrial membrane with an amino-terminal transmembrane domain, while the majority of the protein is extruding into the intermembrane space. (C) 1997 Academic Press.
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Gieffers C, Korioth F, Heimann P, Ungermann C, Frey J. Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. EXPERIMENTAL CELL RESEARCH. 1997;232(2):395-399.
Gieffers, C., Korioth, F., Heimann, P., Ungermann, C., & Frey, J. (1997). Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. EXPERIMENTAL CELL RESEARCH, 232(2), 395-399.
Gieffers, C., Korioth, F., Heimann, P., Ungermann, C., and Frey, J. (1997). Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. EXPERIMENTAL CELL RESEARCH 232, 395-399.
Gieffers, C., et al., 1997. Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. EXPERIMENTAL CELL RESEARCH, 232(2), p 395-399.
C. Gieffers, et al., “Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms”, EXPERIMENTAL CELL RESEARCH, vol. 232, 1997, pp. 395-399.
Gieffers, C., Korioth, F., Heimann, P., Ungermann, C., Frey, J.: Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms. EXPERIMENTAL CELL RESEARCH. 232, 395-399 (1997).
Gieffers, C, Korioth, F, Heimann, Peter, Ungermann, C, and Frey, Jürgen. “Mitofilin is a transmembrane protein of the inner mitochondrial membrane expressed as two isoforms”. EXPERIMENTAL CELL RESEARCH 232.2 (1997): 395-399.

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PMID: 30259514
Potential Role of Mic60/Mitofilin in Parkinson's Disease.
Van Laar VS, Otero PA, Hastings TG, Berman SB., Front Neurosci 12(), 2018
PMID: 30740041
Mitochondrial inner membrane protein (mitofilin) knockdown induces cell death by apoptosis via an AIF-PARP-dependent mechanism and cell cycle arrest.
Madungwe NB, Feng Y, Lie M, Tombo N, Liu L, Kaya F, Bopassa JC., Am J Physiol Cell Physiol 315(1), 2018
PMID: 29489384
The MICOS complex of human mitochondria.
Kozjak-Pavlovic V., Cell Tissue Res 367(1), 2017
PMID: 27245231
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Michaud M, Gros V, Tardif M, Brugière S, Ferro M, Prinz WA, Toulmay A, Mathur J, Wozny M, Falconet D, Maréchal E, Block MA, Jouhet J., Curr Biol 26(5), 2016
PMID: 26898467
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Piñero-Martos E, Ortega-Vila B, Pol-Fuster J, Cisneros-Barroso E, Ruiz-Guerra L, Medina-Dols A, Heine-Suñer D, Lladó J, Olmos G, Vives-Bauzà C., Hum Mol Genet 25(19), 2016
PMID: 27466199
Desmin and αB-crystallin interplay in the maintenance of mitochondrial homeostasis and cardiomyocyte survival.
Diokmetzidou A, Soumaka E, Kloukina I, Tsikitis M, Makridakis M, Varela A, Davos CH, Georgopoulos S, Anesti V, Vlahou A, Capetanaki Y., J Cell Sci 129(20), 2016
PMID: 27566162
OPA1 functionally interacts with MIC60 but is dispensable for crista junction formation.
Barrera M, Koob S, Dikov D, Vogel F, Reichert AS., FEBS Lett 590(19), 2016
PMID: 27587279
QIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphology.
Guarani V, McNeill EM, Paulo JA, Huttlin EL, Fröhlich F, Gygi SP, Van Vactor D, Harper JW., Elife 4(), 2015
PMID: 25997101
Transcriptome and proteome dynamics in larvae of the barnacle Balanus Amphitrite from the Red Sea.
Chandramouli KH, Al-Aqeel S, Ryu T, Zhang H, Seridi L, Ghosheh Y, Qian PY, Ravasi T., BMC Genomics 16(), 2015
PMID: 26666348
Uniform nomenclature for the mitochondrial contact site and cristae organizing system.
Pfanner N, van der Laan M, Amati P, Capaldi RA, Caudy AA, Chacinska A, Darshi M, Deckers M, Hoppins S, Icho T, Jakobs S, Ji J, Kozjak-Pavlovic V, Meisinger C, Odgren PR, Park SK, Rehling P, Reichert AS, Sheikh MS, Taylor SS, Tsuchida N, van der Bliek AM, van der Klei IJ, Weissman JS, Westermann B, Zha J, Neupert W, Nunnari J., J Cell Biol 204(7), 2014
PMID: 24687277
Dissecting the cell to nucleus, perinucleus and cytosol.
Shaiken TE, Opekun AR., Sci Rep 4(), 2014
PMID: 24815916
Mitochondrial metabolism transition cooperates with nuclear reprogramming during induced pluripotent stem cell generation.
Liu W, Long Q, Chen K, Li S, Xiang G, Chen S, Liu X, Li Y, Yang L, Dong D, Jiang C, Feng Z, Qin D, Liu X., Biochem Biophys Res Commun 431(4), 2013
PMID: 23333381
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Weber TA, Koob S, Heide H, Wittig I, Head B, van der Bliek A, Brandt U, Mittelbronn M, Reichert AS., PLoS One 8(5), 2013
PMID: 23704930
Changes in vimentin, lamin A/C and mitofilin induce aberrant cell organization in fibroblasts from Fanconi anemia complementation group A (FA-A) patients.
Capanni C, Bruschi M, Columbaro M, Cuccarolo P, Ravera S, Dufour C, Candiano G, Petretto A, Degan P, Cappelli E., Biochimie 95(10), 2013
PMID: 23831462
CHCM1/CHCHD6, novel mitochondrial protein linked to regulation of mitofilin and mitochondrial cristae morphology.
An J, Shi J, He Q, Lui K, Liu Y, Huang Y, Sheikh MS., J Biol Chem 287(10), 2012
PMID: 22228767
Sam50 functions in mitochondrial intermembrane space bridging and biogenesis of respiratory complexes.
Ott C, Ross K, Straub S, Thiede B, Götz M, Goosmann C, Krischke M, Mueller MJ, Krohne G, Rudel T, Kozjak-Pavlovic V., Mol Cell Biol 32(6), 2012
PMID: 22252321
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Azimzadeh O, Scherthan H, Yentrapalli R, Barjaktarovic Z, Ueffing M, Conrad M, Neff F, Calzada-Wack J, Aubele M, Buske C, Atkinson MJ, Hauck SM, Tapio S., J Proteomics 75(8), 2012
PMID: 22387116
Spatially distinct mitochondrial populations exhibit different mitofilin levels.
Ferreira RM, Vitorino R, Padrão AI, Moreira-Gonçalves D, Alves RM, Duarte JA, Amado F., Cell Biochem Funct 30(5), 2012
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Head BP, Zulaika M, Ryazantsev S, van der Bliek AM., Mol Biol Cell 22(6), 2011
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Feng LX, Jing CJ, Tang KL, Tao L, Cao ZW, Wu WY, Guan SH, Jiang BH, Yang M, Liu X, Guo DA., Proteomics 11(8), 2011
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(-)-Epicatechin enhances fatigue resistance and oxidative capacity in mouse muscle.
Nogueira L, Ramirez-Sanchez I, Perkins GA, Murphy A, Taub PR, Ceballos G, Villarreal FJ, Hogan MC, Malek MH., J Physiol 589(pt 18), 2011
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Mun JY, Lee TH, Kim JH, Yoo BH, Bahk YY, Koo HS, Han SS., J Cell Physiol 224(3), 2010
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Rossi MN, Carbone M, Mostocotto C, Mancone C, Tripodi M, Maione R, Amati P., J Biol Chem 284(46), 2009
PMID: 19762472
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Lai Y, Chen Y, Watkins SC, Nathaniel PD, Guo F, Kochanek PM, Jenkins LW, Szabó C, Clark RS., J Neurochem 104(6), 2008
PMID: 17996029
Mitofilin and titin as target antigens in melanoma-associated retinopathy.
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John GB, Shang Y, Li L, Renken C, Mannella CA, Selker JM, Rangell L, Bennett MJ, Zha J., Mol Biol Cell 16(3), 2005
PMID: 15647377
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Magi B, Ettorre A, Liberatori S, Bini L, Andreassi M, Frosali S, Neri P, Pallini V, Di Stefano A., Cell Death Differ 11(8), 2004
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Identification of proteins regulated by interferon-alpha in resistant and sensitive malignant melanoma cell lines.
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Extensive temporally regulated reorganization of the lipid raft proteome following T-cell antigen receptor triggering.
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Stimulus-induced behavior in F1 hybrids of seizure-sensitive and seizure-resistant gerbils.
Seto-Ohshima A, Kitajima S, Ito M, Inoue M, Murashima YL, Yamakawa K, Itohara S., Zoolog Sci 20(12), 2003
PMID: 14709808


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