Publications

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

View Dr. Proia's publications on Google Scholar.

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Deletion of GBA in neurons, but not microglia, causes neurodegeneration in a Gaucher mouse model.

Duffy HBD, Byrnes C, Zhu H, Tuymetova G, Lee YT, Platt FM, Proia RL.

JCI Insight (2024 Sep 17) Abstract/Full Text

Sialidase NEU3 action on GM1 ganglioside is neuroprotective in GM1 gangliosidosis.

Allende ML, Lee YT, Byrnes C, Li C, Tuymetova G, Bakir JY, Nicoli ER, James VK, Brodbelt JS, Tifft CJ, Proia RL.

J Lipid Res (2023 Dec) 64:100463. Abstract/Full Text

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

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

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

Additional Publications

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

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

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

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

The Complement Regulator Susd4 Influences Nervous-System Function and Neuronal Morphology in Mice.

Zhu H, Meissner LE, Byrnes C, Tuymetova G, Tifft CJ, Proia RL.

iScience (2020 Mar 27) 23:100957. Abstract/Full Text

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

Human GLB1 knockout cerebral organoids: A model system for testing AAV9-mediated GLB1 gene therapy for reducing GM1 ganglioside storage in GM1 gangliosidosis.

Latour YL, Yoon R, Thomas SE, Grant C, Li C, Sena-Esteves M, Allende ML, Proia RL, Tifft CJ.

Mol Genet Metab Rep (2019 Dec) 21:100513. Abstract/Full Text

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

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

Elevating Endogenous Sphingosine-1-Phosphate (S1P) Levels Improves Endothelial Function and Ameliorates Atherosclerosis in Low Density Lipoprotein Receptor-Deficient (LDL-R-/-) Mice.

Feuerborn R, Besser M, Potì F, Burkhardt R, Weißen-Plenz G, Ceglarek U, Simoni M, Proia RL, Freise H, Nofer JR.

Thromb Haemost (2018 Aug) 118:1470-1480. Abstract/Full Text

S1P₄ Regulates Passive Systemic Anaphylaxis in Mice but Is Dispensable for Canonical IgE-Mediated Responses in Mast Cells.

Kulinski JM, Proia RL, Larson EM, Metcalfe DD, Olivera A.

Int J Mol Sci (2018 Apr 25) 19. Abstract/Full Text

Ablation of PPARγ in subcutaneous fat exacerbates age-associated obesity and metabolic decline.

Xu L, Ma X, Verma NK, Wang D, Gavrilova O, Proia RL, Finkel T, Mueller E.

Aging Cell (2018 Apr) 17. Abstract/Full Text

Cerebral organoids derived from Sandhoff disease-induced pluripotent stem cells exhibit impaired neurodifferentiation.

Allende ML, Cook EK, Larman BC, Nugent A, Brady JM, Golebiowski D, Sena-Esteves M, Tifft CJ, Proia RL.

J Lipid Res (2018 Mar) 59:550-563. Abstract/Full Text

The role of G protein-coupled receptors in lymphoid malignancies.

Nugent A, Proia RL.

Cell Signal (2017 Nov) 39:95-107. Abstract/Full Text

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

IGF2BP1 overexpression causes fetal-like hemoglobin expression patterns in cultured human adult erythroblasts.

de Vasconcellos JF, Tumburu L, Byrnes C, Lee YT, Xu PC, Li M, Rabel A, Clarke BA, Guydosh NR, Proia RL, Miller JL.

Proc Natl Acad Sci U S A (2017 Jul 11) 114:E5664-E5672. Abstract/Full Text

De Novo Sphingolipid Biosynthesis Is Required for Adipocyte Survival and Metabolic Homeostasis.

Alexaki A, Clarke BA, Gavrilova O, Ma Y, Zhu H, Ma X, Xu L, Tuymetova G, Larman BC, Allende ML, Dunn TM, Proia RL.

J Biol Chem (2017 Mar 3) 292:3929-3939. Abstract/Full Text

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

Meta-analysis of rare and common exome chip variants identifies S1PR4 and other loci influencing blood cell traits.

CHARGE Consortium Hematology Working Group.

Nat Genet (2016 Aug) 48:867-76. Abstract/Full Text

Sphingosine-1-phosphate Phosphatase 2 Regulates Pancreatic Islet β-Cell Endoplasmic Reticulum Stress and Proliferation.

Taguchi Y, Allende ML, Mizukami H, Cook EK, Gavrilova O, Tuymetova G, Clarke BA, Chen W, Olivera A, Proia RL.

J Biol Chem (2016 Jun 3) 291:12029-38. Abstract/Full Text

The GM1 and GM2 Gangliosidoses: Natural History and Progress toward Therapy.

Regier DS, Proia RL, D'Azzo A, Tifft CJ.

Pediatr Endocrinol Rev (2016 Jun) 13 Suppl 1:663-73. Abstract/Full Text

Sphingosine kinases are not required for inflammatory responses in macrophages.

Xiong Y, Lee HJ, Mariko B, Lu YC, Dannenberg AJ, Haka AS, Maxfield FR, Camerer E, Proia RL, Hla T.

J Biol Chem (2016 May 20) 291:11465. Abstract/Full Text

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

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

Imaging S1P1 activation in vivo.

Kono M, Proia RL.

Exp Cell Res (2015 May 1) 333:178-182. Abstract/Full Text

Activated platelets release sphingosine 1-phosphate and induce hypersensitivity to noxious heat stimuli in vivo.

Weth D, Benetti C, Rauch C, Gstraunthaler G, Schmidt H, Geisslinger G, Sabbadini R, Proia RL, Kress M.

Front Neurosci (2015) 9:140. Abstract/Full Text

Expression of the ORMDLS, modulators of serine palmitoyltransferase, is regulated by sphingolipids in mammalian cells.

Gupta SD, Gable K, Alexaki A, Chandris P, Proia RL, Dunn TM, Harmon JM.

J Biol Chem (2015 Jan 2) 290:90-8. Abstract/Full Text

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

Simplifying complexity: genetically resculpting glycosphingolipid synthesis pathways in mice to reveal function.

Allende ML, Proia RL.

Glycoconj J (2014 Dec) 31:613-22. Abstract/Full Text

Erythrocyte-derived sphingosine 1-phosphate is essential for vascular development.

Xiong Y, Yang P, Proia RL, Hla T.

J Clin Invest (2014 Nov) 124:4823-8. Abstract/Full Text

Essential roles of neutral ceramidase and sphingosine in mitochondrial dysfunction due to traumatic brain injury.

Novgorodov SA, Riley CL, Yu J, Borg KT, Hannun YA, Proia RL, Kindy MS, Gudz TI.

J Biol Chem (2014 May 9) 289:13142-54. Abstract/Full Text

Visualizing S1P-directed cellular egress by intravital imaging.

Giannouli CC, Chandris P, Proia RL.

Biochim Biophys Acta (2014 May) 1841:738-44. Abstract/Full Text

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

Sphingosine kinases are not required for inflammatory responses in macrophages.

Xiong Y, Lee HJ, Mariko B, Lu YC, Dannenberg AJ, Haka AS, Maxfield FR, Camerer E, Proia RL, Hla T.

J Biol Chem (2013 Nov 8) 288:32563-32573. Abstract/Full Text

Sphingosine-1-phosphate can promote mast cell hyper-reactivity through regulation of contactin-4 expression.

Olivera A, Kitamura Y, Wright LD, Allende ML, Chen W, Kaneko-Goto T, Yoshihara Y, Proia RL, Rivera J.

J Leukoc Biol (2013 Nov) 94:1013-24. Abstract/Full Text

Sphingosine-1-phosphate phosphatase 1 regulates keratinocyte differentiation and epidermal homeostasis.

Allende ML, Sipe LM, Tuymetova G, Wilson-Henjum KL, Chen W, Proia RL.

J Biol Chem (2013 Jun 21) 288:18381-91. Abstract/Full Text

Usage of sphingosine kinase isoforms in mast cells is species and/or cell type determined.

Dillahunt SE, Sargent JL, Suzuki R, Proia RL, Gilfillan A, Rivera J, Olivera A.

J Immunol (2013 Mar 1) 190:2058-67. Abstract/Full Text

Sphingosine-1-phosphate-induced nociceptor excitation and ongoing pain behavior in mice and humans is largely mediated by S1P3 receptor.

Camprubí-Robles M, Mair N, Andratsch M, Benetti C, Beroukas D, Rukwied R, Langeslag M, Proia RL, Schmelz M, Ferrer Montiel AV, Haberberger RV, Kress M.

J Neurosci (2013 Feb 6) 33:2582-92. Abstract/Full Text

Shaping the landscape: metabolic regulation of S1P gradients.

Olivera A, Allende ML, Proia RL.

Biochim Biophys Acta (2013 Jan) 1831:193-202. Abstract/Full Text

A novel role of sphingosine 1-phosphate receptor S1pr1 in mouse thrombopoiesis.

Zhang L, Orban M, Lorenz M, Barocke V, Braun D, Urtz N, Schulz C, von Brühl ML, Tirniceriu A, Gaertner F, Proia RL, Graf T, Bolz SS, Montanez E, Prinz M, Müller A, von Baumgarten L, Billich A, Sixt M, Fässler R, von Andrian UH, Junt T, Massberg S.

J Exp Med (2012 Nov 19) 209:2165-81. Abstract/Full Text

B cell-specific S1PR1 deficiency blocks prion dissemination between secondary lymphoid organs.

Mok SW, Proia RL, Brinkmann V, Mabbott NA.

J Immunol (2012 May 15) 188:5032-40. Abstract/Full Text

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

Sphingosine-1-phosphate enhances satellite cell activation in dystrophic muscles through a S1PR2/STAT3 signaling pathway.

Loh KC, Leong WI, Carlson ME, Oskouian B, Kumar A, Fyrst H, Zhang M, Proia RL, Hoffman EP, Saba JD.

PLoS One (2012) 7:e37218. Abstract/Full Text

The sphingosine 1-phosphate receptor S1P₂ maintains the homeostasis of germinal center B cells and promotes niche confinement.

Green JA, Suzuki K, Cho B, Willison LD, Palmer D, Allen CD, Schmidt TH, Xu Y, Proia RL, Coughlin SR, Cyster JG.

Nat Immunol (2011 Jun 5) 12:672-80. Abstract/Full Text

Neurobiological effects of sphingosine 1-phosphate receptor modulation in the cuprizone model.

Kim HJ, Miron VE, Dukala D, Proia RL, Ludwin SK, Traka M, Antel JP, Soliven B.

FASEB J (2011 May) 25:1509-18. Abstract/Full Text