Steven M Claypool

Associate Professor

19992019
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Research Output 1999 2019

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2019

Phosphatidylethanolamine made in the inner mitochondrial membrane is essential for yeast cytochrome bc 1 complex function

Calzada, E., Avery, E., Sam, P. N., Modak, A., Wang, C., McCaffery, J. M., Han, X., Alder, N. N. & Claypool, S. M., Dec 1 2019, In : Nature communications. 10, 1, 1432.

Research output: Contribution to journalArticle

Electron Transport Complex III
cytochromes
yeast
Mitochondrial Membranes
Cytochromes

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

Care4Rare Canada Consortium, Jan 1 2019, In : Life Science Alliance. 2, 2, e201900353.

Research output: Contribution to journalArticle

Open Access
Mitochondrial Diseases
phosphatidylserines
Mitochondrial Genes
cataract
Cataract

Regulation of mitochondrial fragmentation in microvascular endothelial cells isolated from the SU5416/hypoxia model of pulmonary arterial hypertension

Suresh, K., Servinsky, L., Jiang, H., Bigham, Z., Zaldumbide, J., Huetsch, J. C., Kliment, C., Acoba, M. G., Kirsch, B. J., Claypool, S. M., Le, A., Damarla, M. & Shimoda, L. A., Nov 1 2019, In : American journal of physiology. Lung cellular and molecular physiology. 317, 5, p. L639-L652

Research output: Contribution to journalArticle

Open Access
Pulmonary Hypertension
Endothelial Cells
TRPV Cation Channels
Semaxinib
Hypoxia

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

Gokhale, A., Hartwig, C., Freeman, A. A. H., Bassell, J. L., Zlatic, S. A., Sapp Savas, C., Vadlamudi, T., Abudulai, F., Pham, T. T., Crocker, A., Werner, E., Wen, Z., Repetto, G. M., Gogos, J. A., Claypool, S. M., Forsyth, J. K., Bearden, C. E., Glausier, J., Lewis, D. A., Seyfried, N. T. & 2 others, Kwong, J. Q. & Faundez, V., May 1 2019, In : The Journal of neuroscience : the official journal of the Society for Neuroscience. 39, 18, p. 3561-3581 21 p.

Research output: Contribution to journalArticle

Systems Analysis
Synapses
Schizophrenia
Gene Dosage
Membrane Transport Proteins

The Mitochondrial Transacylase, Tafazzin, Regulates for AML Stemness by Modulating Intracellular Levels of Phospholipids

Seneviratne, A. K., Xu, M., Henao, J. J. A., Fajardo, V. A., Hao, Z., Voisin, V., Xu, G. W., Hurren, R., Kim, S., MacLean, N., Wang, X., Gronda, M., Jeyaraju, D., Jitkova, Y., Ketela, T., Mullokandov, M., Sharon, D., Thomas, G., Chouinard-Watkins, R., Hawley, J. R. & 16 others, Schafer, C., Yau, H. L., Khuchua, Z., Aman, A., Al-awar, R., Gross, A., Claypool, S. M., Bazinet, R., Lupien, M., Chan, S., De Carvalho, D. D., Minden, M. D., Bader, G. D., Stark, K. D., LeBlanc, P. & Schimmer, A. D., Apr 4 2019, In : Cell stem cell. 24, 4, p. 621-636.e16

Research output: Contribution to journalArticle

Clustered Regularly Interspaced Short Palindromic Repeats
Acute Myeloid Leukemia
Phospholipids
Cardiolipins
Toll-Like Receptors
2018

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

Ogunbona, O. B., Baile, M. G. & Claypool, S. M., Jun 15 2018, In : Molecular Biology of the Cell. 29, 12, p. 1449-1464 16 p.

Research output: Contribution to journalArticle

ATP Translocases Mitochondrial ADP
Mitochondrial Genome
Electron Transport Complex IV
Cardiomyopathies
Mutation

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, S. Q., Ying, M., Zhang, Y. A., Claypool, S. M., Watkins, P. A. & Xia, S., Jan 1 2018, In : Journal of Biological Chemistry. 293, 17, p. 6544-6555 12 p.

Research output: Contribution to journalArticle

Mitochondrial Dynamics
Glioblastoma
Metabolism
Fusion reactions
Cells
2017

Human adenine nucleotide translocases physically and functionally interact with respirasomes

Lu, Y. W., Acoba, M. G., Selvaraju, K., Huang, T. C., Nirujogi, R. S., Sathe, G., Pandey, A. & Claypool, S. M., Jun 1 2017, In : Molecular Biology of the Cell. 28, 11, p. 1489-1506 18 p.

Research output: Contribution to journalArticle

ATP Translocases Mitochondrial ADP
Oxidative Phosphorylation
Protein Isoforms
Adenosine Diphosphate
Adenosine Triphosphate

Multitiered and cooperative surveillance of mitochondrial phosphatidylserine decarboxylase 1

Ogunbona, O. B., Onguka, O., Calzada, E. & Claypool, S. M., Sep 1 2017, In : Molecular and Cellular Biology. 37, 17, e00049-17.

Research output: Contribution to journalArticle

Peptide Hydrolases
Quality Control
Membranes
Phosphatidylserines
Mitochondrial Membranes

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

Steffen, J., Vashisht, A. A., Wan, J., Jen, J. C., Claypool, S. M., Wohlschlegel, J. A. & Koehler, C. M., Mar 1 2017, In : Molecular Biology of the Cell. 28, 5, p. 600-612 13 p.

Research output: Contribution to journalArticle

Proteasome Endopeptidase Complex
Mitochondrial Degradation
Ubiquitin
Mitochondrial Dynamics
Mitochondria
2016

Defining functional classes of Barth syndrome mutation in humans

Lu, Y. W., Galbraith, L., Herndon, J. D., Lu, Y. L., Pras-Raves, M., Vervaart, M., Van Kampen, A., Luyf, A., Koehler, C. M., McCaffery, J. M., Gottlieb, E., Vaz, F. M. & Claypool, S. M., May 1 2016, In : Human Molecular Genetics. 25, 9, p. 1754-1770 17 p., ddw046.

Research output: Contribution to journalArticle

Barth Syndrome
Yeasts
Mutation
Protein Isoforms
Alleles

Impaired cardiolipin biosynthesis prevents hepatic steatosis and diet-induced obesity

Cole, L. K., Mejia, E. M., Vandel, M., Sparagna, G. C., Claypool, S. M., Dyck-Chan, L., Klein, J. & Hatch, G. M., Nov 1 2016, In : Diabetes. 65, 11, p. 3289-3300 12 p.

Research output: Contribution to journalArticle

Cardiolipins
Obesity
Diet
Liver
Insulin Resistance

Natural and induced mitochondrial phosphate carrier loss: Differential dependence of mitochondrial metabolism and dynamics and cell survival on the extent of depletion

Seifert, E. L., Gál, A., Acoba, M. G., Li, Q., Anderson-Pullinger, L., Golenár, T., Moffat, C., Sondheimer, N., Claypool, S. M. & Hajnóczky, G., Dec 9 2016, In : Journal of Biological Chemistry. 291, 50, p. 26126-26137 12 p.

Research output: Contribution to journalArticle

Phosphate Transport Proteins
Mitochondrial Dynamics
Oxidative Phosphorylation
Fibroblasts
Metabolism

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

Pokorná, L., Čermáková, P., Horváth, A., Baile, M. G., Claypool, S. M., Griač, P., Malínský, J. & Balážová, M., Jan 1 2016, In : Biochimica et Biophysica Acta - Bioenergetics. 1857, 1, p. 34-45 12 p.

Research output: Contribution to journalArticle

Phosphatidylglycerols
Cardiolipins
Yeast
Degradation
Mitochondria

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

Bowman, C. E., Rodriguez, S., Selen Alpergin, E. S., Acoba, M. G., Zhao, L., Hartung, T., Claypool, S. M., Watkins, P. A. & Wolfgang, M. J., Nov 10 2016, (Accepted/In press) In : Cell Chemical Biology.

Research output: Contribution to journalArticle

Malonyl Coenzyme A
Mitochondrial Proteins
Metabolism
Detoxification
Metabolites
2015

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, O. R., Han, X., Hirschey, M. D., Claypool, S. M. & Seifert, E. L., Jan 1 2015, In : Journal of Lipid Research. 55, 12, p. 2458-2470 13 p., A3.

Research output: Contribution to journalArticle

Acyl Coenzyme A
Liver
Fatty Acids
Oxidation
Mitochondria
Mitochondrial Diseases
Mitochondrial Dynamics
Phospholipids
Mitochondrial Genome
Mitochondrial Proteins

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

Bohovych, I., Fernandez, M. R., Rahn, J. J., Stackley, K. D., Bestman, J. E., Anandhan, A., Franco, R., Claypool, S. M., Lewis, R. E., Chan, S. S. L. & Khalimonchuk, O., Sep 14 2015, In : Scientific Reports. 5, 13989.

Research output: Contribution to journalArticle

Metalloproteases
Energy Metabolism
Quality Control
Peptide Hydrolases
Zebrafish

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

Onguka, O., Calzada, E., Ogunbona, O. B. & Claypool, S. M., May 15 2015, In : Journal of Biological Chemistry. 290, 20, p. 12744-12752 9 p.

Research output: Contribution to journalArticle

Membranes
Yeast
Phospholipids
Yeasts
Mitochondrial Dynamics

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

Xu, Y., Malhotra, A., Claypool, S. M., Ren, M. & Schlame, M., Mar 1 2015, In : Mitochondrion. 21, p. 27-32 6 p.

Research output: Contribution to journalArticle

Cardiolipins
Drosophila
Half-Life
Barth Syndrome
Phospholipases A2
2014

The topology and regulation of cardiolipin biosynthesis and remodeling in yeast

Baile, M. G., Lu, Y. W. & Claypool, S. M., 2014, In : Chemistry and Physics of Lipids. 179, p. 25-31 7 p.

Research output: Contribution to journalArticle

Cardiolipins
Biosynthesis
Yeast
Yeasts
Topology

Unremodeled and remodeled cardiolipin are functionally indistinguishable in yeast

Baile, M. G., Sathappa, M., Lu, Y. W., Pryce, E., Whited, K., McCaffery, J. M., Han, X., Alder, N. N. & Claypool, S. M., Jan 17 2014, In : Journal of Biological Chemistry. 289, 3, p. 1768-1778 11 p.

Research output: Contribution to journalArticle

Cardiolipins
Yeast
Yeasts
Tissue
Chemical analysis
2013

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

Baile, M. G., Whited, K. & Claypool, S. M., Jun 15 2013, In : Molecular Biology of the Cell. 24, 12, p. 2008-2020 13 p.

Research output: Contribution to journalArticle

Cardiolipins
Mitochondrial Membranes
Membranes
Barth Syndrome
Mitochondrial Membrane Potential

Mitochondria influence CDR1 efflux pump activity, Hog1-mediated oxidative stress pathway, iron homeostasis, and ergosterol levels in Candida albicans

Thomas, E., Roman, E., Claypool, S. M., Manzoor, N., Pla, J. & Panwar, S. L., Nov 2013, In : Antimicrobial Agents and Chemotherapy. 57, 11, p. 5580-5599 20 p.

Research output: Contribution to journalArticle

Ergosterol
Candida albicans
Mitochondria
Oxidative Stress
Homeostasis

Seven functional classes of barth syndrome mutation

Whited, K., Baile, M. G., Currier, P. & Claypool, S. M., Feb 2013, In : Human Molecular Genetics. 22, 3, p. 483-492 10 p., dds447.

Research output: Contribution to journalArticle

Barth Syndrome
Mutation
Yeasts
Missense Mutation
Cardiomyopathies

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

Baile, M. G. & Claypool, S. M., Jan 1 2013, In : Frontiers in bioscience : a journal and virtual library. 18, 1, p. 241-278 38 p.

Research output: Contribution to journalArticle

Mitochondrial Diseases
Yeast
Yeasts
Physiological Phenomena
Mitochondria

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

Herndon, J. D., Claypool, S. M. & Koehler, C. M., 2013, In : Eukaryotic Cell. 12, 12, p. 1600-1608 9 p.

Research output: Contribution to journalArticle

Mitochondrial Membranes
Membranes
Barth Syndrome
Mutation
Cardiolipins
2012

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

Claypool, S. M., Oktay, Y., Boontheung, P., Loo, J. A. & Koehler, C. M., Jun 25 2012, In : Journal of Cell Biology. 197, 7, p. 1029 1 p.

Research output: Contribution to journalArticle

ATP Translocases Mitochondrial ADP
Cardiolipins
Mitochondrial Membranes
Carrier Proteins
Mitochondria
Phosphatidylserines
Biosynthesis
Proteins
Endoplasmic Reticulum

Role for two conserved intermembrane space proteins, Ups1p and Up2p, in intra-mitochondrial phospholipid trafficking

Tamura, Y., Onguka, O., Aiken Hobbs, A. E., Jensen, R. E., Iijima, M., Claypool, S. M. & Sesaki, H., May 4 2012, In : Journal of Biological Chemistry. 287, 19, p. 15205-15218 14 p.

Research output: Contribution to journalArticle

Mitochondria
Phospholipids
Mitochondrial Membranes
Endoplasmic Reticulum
Proteins
Phospholipids
Proteins

The complexity of cardiolipin in health and disease

Claypool, S. M. & Koehler, C. M., Jan 2012, In : Trends in Biochemical Sciences. 37, 1, p. 32-41 10 p.

Research output: Contribution to journalArticle

Cardiolipins
Health
Metabolism
Biosynthesis
Mitochondria
2011

Barth syndrome mutations that cause tafazzin complex lability

Claypool, S. M., Whited, K., Srijumnong, S., Han, X. & Koehler, C. M., Feb 7 2011, In : Journal of Cell Biology. 192, 3, p. 447-462 16 p.

Research output: Contribution to journalArticle

Barth Syndrome
Mutation
Peptide Hydrolases
Cardiolipins
Missense Mutation
2009

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

Claypool, S. M., Oct 2009, In : BBA - Biomembranes. 1788, 10, p. 2059-2068 10 p.

Research output: Contribution to journalArticle

Cardiolipins
ATP Translocases Mitochondrial ADP
Mitochondrial Proteins
Carrier Proteins
Phospholipids

N-glycan moieties in neonatal Fc receptor determine steady-state membrane distribution and directional transport of IgG

Kuo, T. T., de Muinck, E. J., Claypool, S. M., Yoshida, M., Nagaishi, T., Aveson, V. G., Lencer, W. I. & Blumberg, R. S., Mar 27 2009, In : Journal of Biological Chemistry. 284, 13, p. 8292-8300 9 p.

Research output: Contribution to journalArticle

Polysaccharides
Immunoglobulin G
Membranes
Rats
Epithelial Cells
2008

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

Dickinson, B. L., Claypool, S. M., D'Angelo, J. A., Aiken, M. L., Venu, N., Yen, E. H., Wagner, J. S., Borawski, J. A., Pierce, A. T., Hershberg, R., Blumberg, R. S. & Lencer, W. I., Jan 2008, In : Molecular Biology of the Cell. 19, 1, p. 414-423 10 p.

Research output: Contribution to journalArticle

Transcytosis
Calmodulin
Immunoglobulin G
Mucosal Immunity
Fc Receptors

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

Claypool, S. M., Oktay, Y., Boontheung, P., Loo, J. A. & Koehler, C. M., Sep 8 2008, In : Journal of Cell Biology. 182, 5, p. 937-950 14 p.

Research output: Contribution to journalArticle

ATP Translocases Mitochondrial ADP
Cardiolipins
Mitochondrial Membranes
Carrier Proteins
Chronic Progressive External Ophthalmoplegia

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

Claypool, S. M., Boontheung, P., McCaffery, J. M., Loo, J. A. & Koehler, C. M., Dec 2008, In : Molecular Biology of the Cell. 19, 12, p. 5143-5155 13 p.

Research output: Contribution to journalArticle

Barth Syndrome
Cardiolipins
Adenosine Triphosphate
Mitochondria
Yeasts
2007

Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion

Hwang, D. K., Claypool, S. M., Leuenberger, D., Tienson, H. L. & Koehler, C. M., Sep 24 2007, In : Journal of Cell Biology. 178, 7, p. 1161-1175 15 p.

Research output: Contribution to journalArticle

Mitochondria
Membranes
Yeasts
ATP Translocases Mitochondrial ADP
Mitochondrial DNA
2006

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

Claypool, S. M., McCaffery, J. M. & Koehler, C. M., Jul 31 2006, In : Journal of Cell Biology. 174, 3, p. 379-390 12 p.

Research output: Contribution to journalArticle

Barth Syndrome
Membranes
Mitochondrial Membranes
Mitochondria
Mutation

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

Yoshida, M., Kobayashi, K., Kuo, T. T., Bry, L., Glickman, J. N., Claypool, S. M., Kaser, A., Nagaishi, T., Higgins, D. E., Mizoguchi, E., Wakatsuki, Y., Roopenian, D. C., Mizoguchi, A., Lencer, W. I. & Blumberg, R. S., Aug 1 2006, In : Journal of Clinical Investigation. 116, 8, p. 2142-2151 10 p.

Research output: Contribution to journalArticle

Mucosal Immunity
Immunoglobulin G
Bacteria
Citrobacter rodentium
Epithelium
2005

Characterization of the porcine neonatal Fc receptor - Potential use for trans-epithelial protein delivery

Stirling, C. M. A., Charleston, B., Takamatsu, H., Claypool, S. M., Lencer, W., Blumberg, R. S. & Wileman, T. E., Apr 2005, In : Immunology. 114, 4, p. 542-553 12 p.

Research output: Contribution to journalArticle

Swine
Immunoglobulin G
Proteins
Immunoglobulins
Immune System

Hereditary spastic paraplegia: Respiratory choke or unactivated substrate?

Claypool, S. M. & Koehler, C. M., Oct 21 2005, In : Cell. 123, 2, p. 183-185 3 p.

Research output: Contribution to journalArticle

Hereditary Spastic Paraplegia
Proteolysis
Electric inductors
Mitochondria
Ribosomal Proteins
2004

Bidirectional Transepithelial IgG Transport by a Strongly Polarized Basolateral Membrane Fcγ-Receptor

Claypool, S. M., Dickinson, B. L., Wagner, J. S., Johansen, F. E., Venu, N., Borawski, J. A., Lencer, W. I. & Blumberg, R. S., Apr 2004, In : Molecular Biology of the Cell. 15, 4, p. 1746-1759 14 p.

Research output: Contribution to journalArticle

Fc Receptors
Endocytosis
tryptophan-leucine
Tail
Immunoglobulin G

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

Yoshida, M., Claypool, S. M., Wagner, J. S., Mizoguchi, E., Mizoguchi, A., Roopenian, D. C., Lencer, W. I. & Blumberg, R. S., Jun 2004, In : Immunity. 20, 6, p. 769-783 15 p.

Research output: Contribution to journalArticle

Dendritic Cells
Immunoglobulin G
Antigens
neonatal Fc receptors
Respiratory System
2002

Functional reconstitution of human FcRn in Madin-Darby canine kidney cells requires co-expressed human β2-microglobulin

Claypool, S. M., Dickinson, B. L., Yoshida, M., Lencer, W. I. & Blumberg, R. S., Aug 2 2002, In : Journal of Biological Chemistry. 277, 31, p. 28038-28050 13 p.

Research output: Contribution to journalArticle

Madin Darby Canine Kidney Cells
Immunoglobulin G
Carbohydrates
Glycoproteins
Protein Isoforms
2001

The multiple roles of major histocompatibility complex class-I-like molecules in mucosal immune function

Blumberg, R. S., Van De Wal, Y. D., Claypool, S. M., Corazza, N., Dickinson, B., Nieuwenhuis, E., Pitman, R., Spiekermann, G., Zhu, X., Colgan, S. & Lencer, W. I., Jun 2001, In : Acta Odontologica Scandinavica. 59, 3, p. 139-144 6 p.

Research output: Contribution to journalArticle

Major Histocompatibility Complex
HLA Antigens
Genes
Guanine Nucleotide Dissociation Inhibitors
Epithelial Cells
1999

Antigen presentation by intestinal epithelial cells

Blumberg, R. S., Lencer, W. I., Zhu, X., Kim, H. S., Claypool, S. M., Balk, S. P., Saubermann, L. J. & Colgan, S. P., Jun 15 1999, In : Immunology Letters. 69, 1, p. 7-11 5 p.

Research output: Contribution to journalArticle

Histocompatibility Antigens Class II
Antigen Presentation
Intestinal Mucosa
Epithelial Cells

Developmental regulation of TCRδ locus accessibility and expression by the TCRδ enhancer

Monroe, R. J., Sleckman, B. P., Monroe, B. C., Khor, B., Claypool, S. M., Ferrini, R., Davidson, L. & Alt, F. W., May 1999, In : Immunity. 10, 5, p. 503-513 11 p.

Research output: Contribution to journalArticle

T-Cell Antigen Receptor
T-Lymphocytes
Thymocytes
Mutation
Genes