Richard L. Proia, Ph.D.
Photo of Richard Proia.
Scientific Focus Areas: Genetics and Genomics, Molecular Biology and Biochemistry, Neuroscience, Cell Biology


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

View Dr. Proia’s publications on Google Scholar.

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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
A perilous path: the inborn errors of sphingolipid metabolism.
Dunn TM, Tifft CJ, Proia RL.
J Lipid Res (2019 Mar) 60:475-483. Abstract/Full Text

Additional Publications

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
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
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
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
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 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 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
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
Sphingosine-1-phosphate lyase deficiency produces a pro-inflammatory response while impairing neutrophil trafficking.
Allende ML, Bektas M, Lee BG, Bonifacino E, Kang J, Tuymetova G, Chen W, Saba JD, Proia RL.
J Biol Chem (2011 Mar 4) 286:7348-58. Abstract/Full Text
S1P1 receptor directs the release of immature B cells from bone marrow into blood.
Allende ML, Tuymetova G, Lee BG, Bonifacino E, Wu YP, Proia RL.
J Exp Med (2010 May 10) 207:1113-24. Abstract/Full Text
Sphingosine kinase 1 and sphingosine-1-phosphate receptor 2 are vital to recovery from anaphylactic shock in mice.
Olivera A, Eisner C, Kitamura Y, Dillahunt S, Allende L, Tuymetova G, Watford W, Meylan F, Diesner SC, Li L, Schnermann J, Proia RL, Rivera J.
J Clin Invest (2010 May) 120:1429-40. Abstract/Full Text
Sphingosine 1-phosphate lyase deficiency disrupts lipid homeostasis in liver.
Bektas M, Allende ML, Lee BG, Chen W, Amar MJ, Remaley AT, Saba JD, Proia RL.
J Biol Chem (2010 Apr 2) 285:10880-9. Abstract/Full Text
Absence of oligodendroglial glucosylceramide synthesis does not result in CNS myelin abnormalities or alter the dysmyelinating phenotype of CGT-deficient mice.
Saadat L, Dupree JL, Kilkus J, Han X, Traka M, Proia RL, Dawson G, Popko B.
Glia (2010 Mar) 58:391-8. Abstract/Full Text
The receptor S1P1 overrides regulatory T cell-mediated immune suppression through Akt-mTOR.
Liu G, Burns S, Huang G, Boyd K, Proia RL, Flavell RA, Chi H.
Nat Immunol (2009 Jul) 10:769-77. Abstract/Full Text
Subcellular origin of sphingosine 1-phosphate is essential for its toxic effect in lyase-deficient neurons.
Hagen N, Van Veldhoven PP, Proia RL, Park H, Merrill AH Jr, van Echten-Deckert G.
J Biol Chem (2009 Apr 24) 284:11346-53. Abstract/Full Text
Sphingosine-1-phosphate mobilizes osteoclast precursors and regulates bone homeostasis.
Ishii M, Egen JG, Klauschen F, Meier-Schellersheim M, Saeki Y, Vacher J, Proia RL, Germain RN.
Nature (2009 Mar 26) 458:524-8. Abstract/Full Text
The alliance of sphingosine-1-phosphate and its receptors in immunity.
Rivera J, Proia RL, Olivera A.
Nat Rev Immunol (2008 Oct) 8:753-63. Abstract/Full Text
Sphingosine-1-phosphate regulation of mammalian development.
Kono M, Allende ML, Proia RL.
Biochim Biophys Acta (2008 Sep) 1781:435-41. Abstract/Full Text
Sphingosine kinase 1/S1P receptor signaling axis controls glial proliferation in mice with Sandhoff disease.
Wu YP, Mizugishi K, Bektas M, Sandhoff R, Proia RL.
Hum Mol Genet (2008 Aug 1) 17:2257-64. Abstract/Full Text
Last Reviewed August 2023