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Items: 43


PISD is a mitochondrial disease gene causing skeletal dysplasia, cataracts, and white matter changes.

Zhao T, Goedhart CM, Sam PN, Sabouny R, Lingrell S, Cornish AJ, Lamont RE, Bernier FP, Sinasac D, Parboosingh JS; Care4Rare Canada Consortium, Vance JE, Claypool SM, Innes AM, Shutt TE.

Life Sci Alliance. 2019 Mar 11;2(2). pii: e201900353. doi: 10.26508/lsa.201900353. Print 2019 Apr.


Systems Analysis of the 22q11.2 Microdeletion Syndrome Converges on a Mitochondrial Interactome Necessary for Synapse Function and Behavior.

Gokhale A, Hartwig C, Freeman AAH, Bassell JL, Zlatic SA, Sapp Savas C, Vadlamudi T, Abudulai F, Pham TT, Crocker A, Werner E, Wen Z, Repetto GM, Gogos JA, Claypool SM, Forsyth JK, Bearden CE, Glausier J, Lewis DA, Seyfried NT, Kwong JQ, Faundez V.

J Neurosci. 2019 Mar 4. pii: 1983-18. doi: 10.1523/JNEUROSCI.1983-18.2019. [Epub ahead of print]


Emerging Roles in the Biogenesis of Cytochrome c Oxidase for Members of the Mitochondrial Carrier Family.

Ogunbona OB, Claypool SM.

Front Cell Dev Biol. 2019 Jan 31;7:3. doi: 10.3389/fcell.2019.00003. eCollection 2019. Review.


Cardiomyopathy-associated mutation in the ADP/ATP carrier reveals translation-dependent regulation of cytochrome c oxidase activity.

Ogunbona OB, Baile MG, Claypool SM.

Mol Biol Cell. 2018 Jun 15;29(12):1449-1464. doi: 10.1091/mbc.E17-12-0700. Epub 2018 Apr 24.


Krüppel-like factor 4 (KLF4) induces mitochondrial fusion and increases spare respiratory capacity of human glioblastoma cells.

Wang S, Shi X, Wei S, Ma D, Oyinlade O, Lv SQ, Ying M, Zhang YA, Claypool SM, Watkins P, Xia S.

J Biol Chem. 2018 Apr 27;293(17):6544-6555. doi: 10.1074/jbc.RA117.001323. Epub 2018 Mar 5.


Multitiered and Cooperative Surveillance of Mitochondrial Phosphatidylserine Decarboxylase 1.

Ogunbona OB, Onguka O, Calzada E, Claypool SM.

Mol Cell Biol. 2017 Aug 11;37(17). pii: e00049-17. doi: 10.1128/MCB.00049-17. Print 2017 Sep 1.


The Mammalian Malonyl-CoA Synthetase ACSF3 Is Required for Mitochondrial Protein Malonylation and Metabolic Efficiency.

Bowman CE, Rodriguez S, Selen Alpergin ES, Acoba MG, Zhao L, Hartung T, Claypool SM, Watkins PA, Wolfgang MJ.

Cell Chem Biol. 2017 Jun 22;24(6):673-684.e4. doi: 10.1016/j.chembiol.2017.04.009. Epub 2017 May 4.


Human adenine nucleotide translocases physically and functionally interact with respirasomes.

Lu YW, Acoba MG, Selvaraju K, Huang TC, Nirujogi RS, Sathe G, Pandey A, Claypool SM.

Mol Biol Cell. 2017 Jun 1;28(11):1489-1506. doi: 10.1091/mbc.E17-03-0195. Epub 2017 Apr 12.


Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria.

Steffen J, Vashisht AA, Wan J, Jen JC, Claypool SM, Wohlschlegel JA, Koehler CM.

Mol Biol Cell. 2017 Mar 1;28(5):600-612. doi: 10.1091/mbc.E16-07-0545. Epub 2017 Jan 5.


Erratum. Impaired Cardiolipin Biosynthesis Prevents Hepatic Steatosis and Diet-Induced Obesity. Diabetes 2016;65:3289-3300.

Cole LK, Mejia EM, Vandel M, Sparagna GC, Claypool SM, Dyck-Chan L, Klein J, Hatch GM.

Diabetes. 2017 Jan;66(1):231. doi: 10.2337/db17-er01a. Epub 2016 Nov 17. No abstract available.



Seifert EL, Gál A, Acoba MG, Li Q, Anderson-Pullinger L, Golenár T, Moffat C, Sondheimer N, Claypool SM, Hajnóczky G.

J Biol Chem. 2016 Dec 9;291(50):26126-26137. Epub 2016 Oct 25.


Impaired Cardiolipin Biosynthesis Prevents Hepatic Steatosis and Diet-Induced Obesity.

Cole LK, Mejia EM, Vandel M, Sparagna GC, Claypool SM, Dyck-Chan L, Klein J, Hatch GM.

Diabetes. 2016 Nov;65(11):3289-3300. Epub 2016 Aug 5. Erratum in: Diabetes. 2017 Jan;66(1):231.


Defining functional classes of Barth syndrome mutation in humans.

Lu YW, Galbraith L, Herndon JD, Lu YL, Pras-Raves M, Vervaart M, Van Kampen A, Luyf A, Koehler CM, McCaffery JM, Gottlieb E, Vaz FM, Claypool SM.

Hum Mol Genet. 2016 May 1;25(9):1754-70. doi: 10.1093/hmg/ddw046. Epub 2016 Feb 16.


Phosphatidylethanolamine Metabolism in Health and Disease.

Calzada E, Onguka O, Claypool SM.

Int Rev Cell Mol Biol. 2016;321:29-88. doi: 10.1016/bs.ircmb.2015.10.001. Epub 2015 Oct 31. Review.


Specific degradation of phosphatidylglycerol is necessary for proper mitochondrial morphology and function.

Pokorná L, Čermáková P, Horváth A, Baile MG, Claypool SM, Griač P, Malínský J, Balážová M.

Biochim Biophys Acta. 2016 Jan;1857(1):34-45. doi: 10.1016/j.bbabio.2015.10.004. Epub 2015 Oct 19.


Metalloprotease OMA1 Fine-tunes Mitochondrial Bioenergetic Function and Respiratory Supercomplex Stability.

Bohovych I, Fernandez MR, Rahn JJ, Stackley KD, Bestman JE, Anandhan A, Franco R, Claypool SM, Lewis RE, Chan SS, Khalimonchuk O.

Sci Rep. 2015 Sep 14;5:13989. doi: 10.1038/srep13989.


Phosphatidylserine decarboxylase 1 autocatalysis and function does not require a mitochondrial-specific factor.

Onguka O, Calzada E, Ogunbona OB, Claypool SM.

J Biol Chem. 2015 May 15;290(20):12744-52. doi: 10.1074/jbc.M115.641118. Epub 2015 Mar 31.


Disorders of phospholipid metabolism: an emerging class of mitochondrial disease due to defects in nuclear genes.

Lu YW, Claypool SM.

Front Genet. 2015 Feb 3;6:3. doi: 10.3389/fgene.2015.00003. eCollection 2015. Review.


Tafazzins from Drosophila and mammalian cells assemble in large protein complexes with a short half-life.

Xu Y, Malhotra A, Claypool SM, Ren M, Schlame M.

Mitochondrion. 2015 Mar;21:27-32. doi: 10.1016/j.mito.2015.01.002. Epub 2015 Jan 15.


Acyl-CoA thioesterase-2 facilitates mitochondrial fatty acid oxidation in the liver.

Moffat C, Bhatia L, Nguyen T, Lynch P, Wang M, Wang D, Ilkayeva OR, Han X, Hirschey MD, Claypool SM, Seifert EL.

J Lipid Res. 2014 Dec;55(12):2458-70. doi: 10.1194/jlr.M046961. Epub 2014 Aug 11.


Unremodeled and remodeled cardiolipin are functionally indistinguishable in yeast.

Baile MG, Sathappa M, Lu YW, Pryce E, Whited K, McCaffery JM, Han X, Alder NN, Claypool SM.

J Biol Chem. 2014 Jan 17;289(3):1768-78. doi: 10.1074/jbc.M113.525733. Epub 2013 Nov 27.


The topology and regulation of cardiolipin biosynthesis and remodeling in yeast.

Baile MG, Lu YW, Claypool SM.

Chem Phys Lipids. 2014 Apr;179:25-31. doi: 10.1016/j.chemphyslip.2013.10.008. Epub 2013 Nov 1. Review.


The Taz1p transacylase is imported and sorted into the outer mitochondrial membrane via a membrane anchor domain.

Herndon JD, Claypool SM, Koehler CM.

Eukaryot Cell. 2013 Dec;12(12):1600-8. doi: 10.1128/EC.00237-13. Epub 2013 Sep 27.


Deacylation on the matrix side of the mitochondrial inner membrane regulates cardiolipin remodeling.

Baile MG, Whited K, Claypool SM.

Mol Biol Cell. 2013 Jun;24(12):2008-20. doi: 10.1091/mbc.E13-03-0121. Epub 2013 May 1.


The power of yeast to model diseases of the powerhouse of the cell.

Baile MG, Claypool SM.

Front Biosci (Landmark Ed). 2013 Jan 1;18:241-78. Review.


Phosphatidylethanolamine biosynthesis in mitochondria: phosphatidylserine (PS) trafficking is independent of a PS decarboxylase and intermembrane space proteins UPS1P and UPS2P.

Tamura Y, Onguka O, Itoh K, Endo T, Iijima M, Claypool SM, Sesaki H.

J Biol Chem. 2012 Dec 21;287(52):43961-71. doi: 10.1074/jbc.M112.390997. Epub 2012 Nov 2.


Seven functional classes of Barth syndrome mutation.

Whited K, Baile MG, Currier P, Claypool SM.

Hum Mol Genet. 2013 Feb 1;22(3):483-92. doi: 10.1093/hmg/dds447. Epub 2012 Oct 24.


Role for two conserved intermembrane space proteins, Ups1p and Ups2p, [corrected] in intra-mitochondrial phospholipid trafficking.

Tamura Y, Onguka O, Hobbs AE, Jensen RE, Iijima M, Claypool SM, Sesaki H.

J Biol Chem. 2012 May 4;287(19):15205-18. doi: 10.1074/jbc.M111.338665. Epub 2012 Mar 7. Erratum in: J Biol Chem. 2012 Aug 10;287(33):27450.


The complexity of cardiolipin in health and disease.

Claypool SM, Koehler CM.

Trends Biochem Sci. 2012 Jan;37(1):32-41. doi: 10.1016/j.tibs.2011.09.003. Epub 2011 Oct 17. Review.


Barth syndrome mutations that cause tafazzin complex lability.

Claypool SM, Whited K, Srijumnong S, Han X, Koehler CM.

J Cell Biol. 2011 Feb 7;192(3):447-62. doi: 10.1083/jcb.201008177.


Cardiolipin, a critical determinant of mitochondrial carrier protein assembly and function.

Claypool SM.

Biochim Biophys Acta. 2009 Oct;1788(10):2059-68. doi: 10.1016/j.bbamem.2009.04.020. Epub 2009 May 5. Review.


N-Glycan Moieties in Neonatal Fc Receptor Determine Steady-state Membrane Distribution and Directional Transport of IgG.

Kuo TT, de Muinck EJ, Claypool SM, Yoshida M, Nagaishi T, Aveson VG, Lencer WI, Blumberg RS.

J Biol Chem. 2009 Mar 27;284(13):8292-300. doi: 10.1074/jbc.M805877200. Epub 2009 Jan 21.


The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome.

Claypool SM, Boontheung P, McCaffery JM, Loo JA, Koehler CM.

Mol Biol Cell. 2008 Dec;19(12):5143-55. doi: 10.1091/mbc.E08-09-0896. Epub 2008 Sep 17. Erratum in: Mol Biol Cell. 2012 Jul;23(13):2619.


Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane.

Claypool SM, Oktay Y, Boontheung P, Loo JA, Koehler CM.

J Cell Biol. 2008 Sep 8;182(5):937-50. doi: 10.1083/jcb.200801152. Erratum in: J Cell Biol. 2012 Jun 25;197(7):1029.


Ca2+-dependent calmodulin binding to FcRn affects immunoglobulin G transport in the transcytotic pathway.

Dickinson BL, Claypool SM, D'Angelo JA, Aiken ML, Venu N, Yen EH, Wagner JS, Borawski JA, Pierce AT, Hershberg R, Blumberg RS, Lencer WI.

Mol Biol Cell. 2008 Jan;19(1):414-23. Epub 2007 Nov 14.


Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion.

Hwang DK, Claypool SM, Leuenberger D, Tienson HL, Koehler CM.

J Cell Biol. 2007 Sep 24;178(7):1161-75.


IgG transport across mucosal barriers by neonatal Fc receptor for IgG and mucosal immunity.

Yoshida M, Masuda A, Kuo TT, Kobayashi K, Claypool SM, Takagawa T, Kutsumi H, Azuma T, Lencer WI, Blumberg RS.

Springer Semin Immunopathol. 2006 Dec;28(4):397-403. Epub 2006 Oct 19.


Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins.

Claypool SM, McCaffery JM, Koehler CM.

J Cell Biol. 2006 Jul 31;174(3):379-90.


Neonatal Fc receptor for IgG regulates mucosal immune responses to luminal bacteria.

Yoshida M, Kobayashi K, Kuo TT, Bry L, Glickman JN, Claypool SM, Kaser A, Nagaishi T, Higgins DE, Mizoguchi E, Wakatsuki Y, Roopenian DC, Mizoguchi A, Lencer WI, Blumberg RS.

J Clin Invest. 2006 Aug;116(8):2142-2151.


Hereditary spastic paraplegia: respiratory choke or unactivated substrate?

Claypool SM, Koehler CM.

Cell. 2005 Oct 21;123(2):183-5.


Human neonatal Fc receptor mediates transport of IgG into luminal secretions for delivery of antigens to mucosal dendritic cells.

Yoshida M, Claypool SM, Wagner JS, Mizoguchi E, Mizoguchi A, Roopenian DC, Lencer WI, Blumberg RS.

Immunity. 2004 Jun;20(6):769-83.


Bidirectional transepithelial IgG transport by a strongly polarized basolateral membrane Fcgamma-receptor.

Claypool SM, Dickinson BL, Wagner JS, Johansen FE, Venu N, Borawski JA, Lencer WI, Blumberg RS.

Mol Biol Cell. 2004 Apr;15(4):1746-59. Epub 2004 Feb 6.


Functional reconstitution of human FcRn in Madin-Darby canine kidney cells requires co-expressed human beta 2-microglobulin.

Claypool SM, Dickinson BL, Yoshida M, Lencer WI, Blumberg RS.

J Biol Chem. 2002 Aug 2;277(31):28038-50. Epub 2002 May 22.

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