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Mari Kono, Ph.D.
Mari Kono.
Staff Scientist: Genetics of Development and Disease Section, Genetics & Biochemistry Branch
Scientific Focus Areas: Genetics and Genomics, Molecular Biology and Biochemistry, Cell Biology, Neuroscience, Immunology

Publications

A selection of recent and significant publications can be viewed below.

SARS-CoV-2 ORF3a expression in brain disrupts the autophagy-lysosomal pathway, impairs sphingolipid homeostasis, and drives neuropathogenesis.
Zhu H, Byrnes C, Lee YT, Tuymetova G, Duffy HBD, Bakir JY, Pettit SN, Angina J, Springer DA, Allende ML, Kono M, Proia RL.
FASEB J (2023 May) 37:e22919. Abstract/Full Text NIH external link
Visualizing Sphingosine-1-Phosphate Receptor 1(S1P(1)) Signaling During Central Nervous System De- and Remyelination.
Hashemi E, Yoseph E, Tsai HC, Moreno M, Yeh LH, Mehta SB, Kono M, Proia R, Han MH.
Cell Mol Neurobiol (2023 Apr) 43:1219-1236. Abstract/Full Text NIH external link
Identification of two lipid phosphatases that regulate sphingosine-1-phosphate cellular uptake and recycling.
Kono M, Hoachlander-Hobby LE, Majumder S, Schwartz R, Byrnes C, Zhu H, Proia RL.
J Lipid Res (2022 Jun) 63:100225. Abstract/Full Text NIH external link
Endothelial S1P(1) Signaling Counteracts Infarct Expansion in Ischemic Stroke.
Nitzsche A, Poittevin M, Benarab A, Bonnin P, Faraco G, Uchida H, Favre J, Garcia-Bonilla L, Garcia MCL, Léger PL, Thérond P, Mathivet T, Autret G, Baudrie V, Couty L, Kono M, Chevallier A, Niazi H, Tharaux PL, Chun J, Schwab SR, Eichmann A, Tavitian B, Proia RL, Charriaut-Marlangue C, Sanchez T, Kubis N, Henrion D, Iadecola C, Hla T, Camerer E.
Circ Res (2021 Feb 5) 128:363-382. Abstract/Full Text NIH external link
Genetic defects in the sphingolipid degradation pathway and their effects on microglia in neurodegenerative disease.
Allende ML, Zhu H, Kono M, Hoachlander-Hobby LE, Huso VL, Proia RL.
Cell Signal (2021 Feb) 78:109879. Abstract/Full Text NIH external link
A genome-wide CRISPR/Cas9 screen reveals that the aryl hydrocarbon receptor stimulates sphingolipid levels.
Majumder S, Kono M, Lee YT, Byrnes C, Li C, Tuymetova G, Proia RL.
J Biol Chem (2020 Mar 27) 295:4341-4349. Abstract/Full Text NIH external link
Sphingosine 1-phosphate-regulated transcriptomes in heterogenous arterial and lymphatic endothelium of the aorta.
Engelbrecht E, Levesque MV, He L, Vanlandewijck M, Nitzsche A, Niazi H, Kuo A, Singh SA, Aikawa M, Holton K, Proia RL, Kono M, Pu WT, Camerer E, Betsholtz C, Hla T.
Elife (2020 Feb 24) 9. Abstract/Full Text NIH external link
The Ormdl genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice.
Clarke BA, Majumder S, Zhu H, Lee YT, Kono M, Li C, Khanna C, Blain H, Schwartz R, Huso VL, Byrnes C, Tuymetova G, Dunn TM, Allende ML, Proia RL.
Elife (2019 Dec 27) 8. Abstract/Full Text NIH external link
Lysolipid receptor cross-talk regulates lymphatic endothelial junctions in lymph nodes.
Hisano Y, Kono M, Cartier A, Engelbrecht E, Kano K, Kawakami K, Xiong Y, Piao W, Galvani S, Yanagida K, Kuo A, Ono Y, Ishida S, Aoki J, Proia RL, Bromberg JS, Inoue A, Hla T.
J Exp Med (2019 Jul 1) 216:1582-1598. Abstract/Full Text NIH external link
Murine platelet production is suppressed by S1P release in the hematopoietic niche, not facilitated by blood S1P sensing.
Niazi H, Zoghdani N, Couty L, Leuci A, Nitzsche A, Allende ML, Mariko B, Ishaq R, Aslan Y, Becker PH, Gazit SL, Poirault-Chassac S, Decouture B, Baudrie V, De Candia E, Kono M, Benarab A, Gaussem P, Tharaux PL, Chun J, Provot S, Debili N, Therond P, Proia RL, Bachelot-Loza C, Camerer E.
Blood Adv (2019 Jun 11) 3:1702-1713. Abstract/Full Text NIH external link
Bioluminescence imaging of G protein-coupled receptor activation in living mice.
Kono M, Conlon EG, Lux SY, Yanagida K, Hla T, Proia RL.
Nat Commun (2017 Oct 27) 8:1163. Abstract/Full Text NIH external link
Targeting cancer metabolism by simultaneously disrupting parallel nutrient access pathways.
Kim SM, Roy SG, Chen B, Nguyen TM, McMonigle RJ, McCracken AN, Zhang Y, Kofuji S, Hou J, Selwan E, Finicle BT, Nguyen TT, Ravi A, Ramirez MU, Wiher T, Guenther GG, Kono M, Sasaki AT, Weisman LS, Potma EO, Tromberg BJ, Edwards RA, Hanessian S, Edinger AL.
J Clin Invest (2016 Nov 1) 126:4088-4102. Abstract/Full Text NIH external link
HDL-bound sphingosine 1-phosphate acts as a biased agonist for the endothelial cell receptor S1P1 to limit vascular inflammation.
Galvani S, Sanson M, Blaho VA, Swendeman SL, Obinata H, Conger H, Dahlbäck B, Kono M, Proia RL, Smith JD, Hla T.
Sci Signal (2015 Aug 11) 8:ra79. Abstract/Full Text NIH external link
HDL-bound sphingosine-1-phosphate restrains lymphopoiesis and neuroinflammation.
Blaho VA, Galvani S, Engelbrecht E, Liu C, Swendeman SL, Kono M, Proia RL, Steinman L, Han MH, Hla T.
Nature (2015 Jul 16) 523:342-6. Abstract/Full Text NIH external link
Imaging S1P1 activation in vivo.
Kono M, Proia RL.
Exp Cell Res (2015 May 1) 333:178-182. Abstract/Full Text NIH external link
Autophagy regulates sphingolipid levels in the liver.
Alexaki A, Gupta SD, Majumder S, Kono M, Tuymetova G, Harmon JM, Dunn TM, Proia RL.
J Lipid Res (2014 Dec) 55:2521-31. Abstract/Full Text NIH external link
Sphingosine-1-phosphate receptor 1 reporter mice reveal receptor activation sites in vivo.
Kono M, Tucker AE, Tran J, Bergner JB, Turner EM, Proia RL.
J Clin Invest (2014 May) 124:2076-86. Abstract/Full Text NIH external link
Disulphide linkage in mouse ST6Gal-I: determination of linkage positions and mutant analysis.
Hirano Y, Suzuki T, Matsumoto T, Ishihara Y, Takaki Y, Kono M, Dohmae N, Tsuji S.
J Biochem (2012 Feb) 151:197-203. Abstract/Full Text NIH external link
Sphingosine-1-phosphate regulation of mammalian development.
Kono M, Allende ML, Proia RL.
Biochim Biophys Acta (2008 Sep) 1781:435-41. Abstract/Full Text NIH external link
Deafness and stria vascularis defects in S1P2 receptor-null mice.
Kono M, Belyantseva IA, Skoura A, Frolenkov GI, Starost MF, Dreier JL, Lidington D, Bolz SS, Friedman TB, Hla T, Proia RL.
J Biol Chem (2007 Apr 6) 282:10690-6. Abstract/Full Text NIH external link
Depletion of ceramides with very long chain fatty acids causes defective skin permeability barrier function, and neonatal lethality in ELOVL4 deficient mice.
Li W, Sandhoff R, Kono M, Zerfas P, Hoffmann V, Ding BC, Proia RL, Deng CX.
Int J Biol Sci (2007 Feb 6) 3:120-8. Abstract/Full Text NIH external link
Neutral ceramidase encoded by the Asah2 gene is essential for the intestinal degradation of sphingolipids.
Kono M, Dreier JL, Ellis JM, Allende ML, Kalkofen DN, Sanders KM, Bielawski J, Bielawska A, Hannun YA, Proia RL.
J Biol Chem (2006 Mar 17) 281:7324-31. Abstract/Full Text NIH external link
The sphingosine-1-phosphate receptors S1P1, S1P2, and S1P3 function coordinately during embryonic angiogenesis.
Kono M, Mi Y, Liu Y, Sasaki T, Allende ML, Wu YP, Yamashita T, Proia RL.
J Biol Chem (2004 Jul 9) 279:29367-73. Abstract/Full Text NIH external link
Enhanced insulin sensitivity in mice lacking ganglioside GM3.
Yamashita T, Hashiramoto A, Haluzik M, Mizukami H, Beck S, Norton A, Kono M, Tsuji S, Daniotti JL, Werth N, Sandhoff R, Sandhoff K, Proia RL.
Proc Natl Acad Sci U S A (2003 Mar 18) 100:3445-9. Abstract/Full Text NIH external link
Cloning and potential utility of porcine Fas ligand: overexpression in porcine endothelial cells protects them from attack by human cytolytic cells.
Tsuyuki S, Kono M, Bloom ET.
Xenotransplantation (2002 Nov) 9:410-21. Abstract/Full Text NIH external link
Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage.
Mizukami H, Mi Y, Wada R, Kono M, Yamashita T, Liu Y, Werth N, Sandhoff R, Sandhoff K, Proia RL.
J Clin Invest (2002 May) 109:1215-21. Abstract/Full Text NIH external link
Identification and functional characterization of a human GalNAc [alpha]2,6-sialyltransferase with altered expression in breast cancer.
Sotiropoulou G, Kono M, Anisowicz A, Stenman G, Tsuji S, Sager R.
Mol Med (2002 Jan) 8:42-55. Abstract/Full Text NIH external link
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures.
Kawai H, Allende ML, Wada R, Kono M, Sango K, Deng C, Miyakawa T, Crawley JN, Werth N, Bierfreund U, Sandhoff K, Proia RL.
J Biol Chem (2001 Mar 9) 276:6885-8. Abstract/Full Text NIH external link
Genomic organization and transcriptional regulation of the mouse GD3 synthase gene (ST8Sia I): comparison of genomic organization of the mouse sialyltransferase genes.
Takashima S, Kono M, Kurosawa N, Yoshida Y, Tachida Y, Inoue M, Kanematsu T, Tsuji S.
J Biochem (2000 Dec) 128:1033-43. Abstract/Full Text NIH external link
Redefined substrate specificity of ST6GalNAc II: a second candidate sialyl-Tn synthase.
Kono M, Tsuda T, Ogata S, Takashima S, Liu H, Hamamoto T, Itzkowitz SH, Nishimura S, Tsuji S.
Biochem Biophys Res Commun (2000 May 27) 272:94-7. Abstract/Full Text NIH external link
Molecular cloning and genomic analysis of mouse GalNAc alpha2, 6-sialyltransferase (ST6GalNAc I).
Kurosawa N, Takashima S, Kono M, Ikehara Y, Inoue M, Tachida Y, Narimatsu H, Tsuji S.
J Biochem (2000 May) 127:845-54. Abstract/Full Text NIH external link
A novel glycosyltransferase with a polyglutamine repeat; a new candidate for GD1alpha synthase (ST6GalNAc V)(1).
Ikehara Y, Shimizu N, Kono M, Nishihara S, Nakanishi H, Kitamura T, Narimatsu H, Tsuji S, Tatematsu M.
FEBS Lett (1999 Dec 10) 463:92-6. Abstract/Full Text NIH external link
Cloning and expression of a human gene encoding an N-acetylgalactosamine-alpha2,6-sialyltransferase (ST6GalNAc I): a candidate for synthesis of cancer-associated sialyl-Tn antigens.
Ikehara Y, Kojima N, Kurosawa N, Kudo T, Kono M, Nishihara S, Issiki S, Morozumi K, Itzkowitz S, Tsuda T, Nishimura SI, Tsuji S, Narimatsu H.
Glycobiology (1999 Nov) 9:1213-24. Abstract/Full Text NIH external link
Molecular cloning and functional expression of two members of mouse NeuAcalpha2,3Galbeta1,3GalNAc GalNAcalpha2,6-sialyltransferase family, ST6GalNAc III and IV.
Lee YC, Kaufmann M, Kitazume-Kawaguchi S, Kono M, Takashima S, Kurosawa N, Liu H, Pircher H, Tsuji S.
J Biol Chem (1999 Apr 23) 274:11958-67. Abstract/Full Text NIH external link
Molecular cloning and functional expression of a fifth-type alpha 2,3-sialyltransferase (mST3Gal V: GM3 synthase).
Kono M, Takashima S, Liu H, Inoue M, Kojima N, Lee YC, Hamamoto T, Tsuji S.
Biochem Biophys Res Commun (1998 Dec 9) 253:170-5. Abstract/Full Text NIH external link
Mouse beta-galactoside alpha 2,3-sialyltransferases: comparison of in vitro substrate specificities and tissue specific expression.
Kono M, Ohyama Y, Lee YC, Hamamoto T, Kojima N, Tsuji S.
Glycobiology (1997 Jun) 7:469-79. Abstract/Full Text NIH external link
Molecular cloning and expression of a fifth type of alpha2,8-sialyltransferase (ST8Sia V). Its substrate specificity is similar to that of SAT-V/III, which synthesize GD1c, GT1a, GQ1b and GT3.
Kono M, Yoshida Y, Kojima N, Tsuji S.
J Biol Chem (1996 Nov 15) 271:29366-71. Abstract/Full Text NIH external link
Biosynthesis and expression of polysialic acid on the neural cell adhesion molecule is predominantly directed by ST8Sia II/STX during in vitro neuronal differentiation.
Kojima N, Kono M, Yoshida Y, Tachida Y, Nakafuku M, Tsuji S.
J Biol Chem (1996 Sep 6) 271:22058-62. Abstract/Full Text NIH external link
Last Reviewed August 2024