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profileRichard S. Smith

TitleResearch Fellow in Pediatrics (EXT)
InstitutionBoston Children's Hospital
DepartmentPediatrics
AddressChildren's Hospital
300 Longwood Ave
Boston MA 02115
Phone617/919-2785
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Collapse Research 
Collapse research activities and funding
The research activities and funding listed below are automatically derived from NIH ExPORTER and other sources, which might result in incorrect or missing items. Faculty can login to make corrections and additions.
R24EY014751     (SMITH, RICHARD S)Apr 1, 2003 - Mar 31, 2009
NIH/NEI
Histopathology and Electroretinography Services
Role: Principal Investigator

R01EY011837     (SMITH, RICHARD S)Dec 1, 1998 - Nov 30, 2003
NIH/NEI
EPITHELIAL AND ENDOTHELIAL PROLIFERATION IN CORN1 MICE
Role: Principal Investigator

P01RR008911     (SUNDBERG, JOHN P)Sep 30, 1993 - Sep 29, 2000
NIH/NCRR
CORE--PATHOLOGY
Role: Co-Principal Investigator

P30CA034196     (LIU, EDISON TAK-BUN)Aug 1, 1983 - Jun 30, 2019
NIH/NCI
Cancer Center Support (CORE) Grant
Role: Co-Principal Investigator

Collapse Bibliographic 
Collapse selected publications
Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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  1. Harder JM, Braine CE, Williams PA, Zhu X, MacNicoll KH, Sousa GL, Buchanan RA, Smith RS, Libby RT, Howell GR, John SWM. Early immune responses are independent of RGC dysfunction in glaucoma with complement component C3 being protective. Proc Natl Acad Sci U S A. 2017 May 09; 114(19):E3839-E3848. PMID: 28446616.
    View in: PubMed
  2. Nair KS, Cosma M, Raghupathy N, Sellarole MA, Tolman NG, de Vries W, Smith RS, John SW. YBR/EiJ mice: a new model of glaucoma caused by genes on chromosomes 4 and 17. Dis Model Mech. 2016 Aug 01; 9(8):863-71. PMID: 27483353; PMCID: PMC5007977.
  3. Mao M, Smith RS, Alavi MV, Marchant JK, Cosma M, Libby RT, John SW, Gould DB. Strain-Dependent Anterior Segment Dysgenesis and Progression to Glaucoma in Col4a1 Mutant Mice. Invest Ophthalmol Vis Sci. 2015 Oct; 56(11):6823-31. PMID: 26567795; PMCID: PMC4627250 [Available on 04/01/16].
  4. Cross SH, Macalinao DG, McKie L, Rose L, Kearney AL, Rainger J, Thaung C, Keighren M, Jadeja S, West K, Kneeland SC, Smith RS, Howell GR, Young F, Robertson M, van T' Hof R, John SW, Jackson IJ. A dominant-negative mutation of mouse Lmx1b causes glaucoma and is semi-lethal via LDB1-mediated dimerization [corrected]. PLoS Genet. 2014 May; 10(5):e1004359. PMID: 24809698; PMCID: PMC4014447.
  5. Nair KS, Barbay J, Smith RS, Masli S, John SW. Determining immune components necessary for progression of pigment dispersing disease to glaucoma in DBA/2J mice. BMC Genet. 2014 Mar 28; 15:42. PMID: 24678736; PMCID: PMC3974199.
  6. Coleman JA, Zhu X, Djajadi HR, Molday LL, Smith RS, Libby RT, John SW, Molday RS. Phospholipid flippase ATP8A2 is required for normal visual and auditory function and photoreceptor and spiral ganglion cell survival. J Cell Sci. 2014 Mar 01; 127(Pt 5):1138-49. PMID: 24413176; PMCID: PMC3937779.
  7. Howell GR, Soto I, Ryan M, Graham LC, Smith RS, John SW. Deficiency of complement component 5 ameliorates glaucoma in DBA/2J mice. J Neuroinflammation. 2013 Jun 27; 10:76. PMID: 23806181; PMCID: PMC3708765.
  8. Zhu X, Libby RT, de Vries WN, Smith RS, Wright DL, Bronson RT, Seburn KL, John SW. Mutations in a P-type ATPase gene cause axonal degeneration. PLoS Genet. 2012; 8(8):e1002853. PMID: 22912588; PMCID: PMC3415440.
  9. Howell GR, Soto I, Zhu X, Ryan M, Macalinao DG, Sousa GL, Caddle LB, MacNicoll KH, Barbay JM, Porciatti V, Anderson MG, Smith RS, Clark AF, Libby RT, John SW. Radiation treatment inhibits monocyte entry into the optic nerve head and prevents neuronal damage in a mouse model of glaucoma. J Clin Invest. 2012 Apr; 122(4):1246-61. PMID: 22426214; PMCID: PMC3314470.
  10. Nair KS, Hmani-Aifa M, Ali Z, Kearney AL, Ben Salem S, Macalinao DG, Cosma IM, Bouassida W, Hakim B, Benzina Z, Soto I, Söderkvist P, Howell GR, Smith RS, Ayadi H, John SW. Alteration of the serine protease PRSS56 causes angle-closure glaucoma in mice and posterior microphthalmia in humans and mice. Nat Genet. 2011 Jun; 43(6):579-84. PMID: 21532570; PMCID: PMC4388060.
  11. Lachke SA, Alkuraya FS, Kneeland SC, Ohn T, Aboukhalil A, Howell GR, Saadi I, Cavallesco R, Yue Y, Tsai AC, Nair KS, Cosma MI, Smith RS, Hodges E, Alfadhli SM, Al-Hajeri A, Shamseldin HE, Behbehani A, Hannon GJ, Bulyk ML, Drack AV, Anderson PJ, John SW, Maas RL. Mutations in the RNA granule component TDRD7 cause cataract and glaucoma. Science. 2011 Mar 25; 331(6024):1571-6. PMID: 21436445; PMCID: PMC3279122.
  12. Veth KN, Willer JR, Collery RF, Gray MP, Willer GB, Wagner DS, Mullins MC, Udvadia AJ, Smith RS, John SW, Gregg RG, Link BA. Mutations in zebrafish lrp2 result in adult-onset ocular pathogenesis that models myopia and other risk factors for glaucoma. PLoS Genet. 2011 Feb; 7(2):e1001310. PMID: 21379331; PMCID: PMC3040661.
  13. Won J, Marín de Evsikova C, Smith RS, Hicks WL, Edwards MM, Longo-Guess C, Li T, Naggert JK, Nishina PM. NPHP4 is necessary for normal photoreceptor ribbon synapse maintenance and outer segment formation, and for sperm development. Hum Mol Genet. 2011 Feb 01; 20(3):482-96. PMID: 21078623; PMCID: PMC3016909.
  14. Lee Y, Smith RS, Jordan W, King BL, Won J, Valpuesta JM, Naggert JK, Nishina PM. Prefoldin 5 is required for normal sensory and neuronal development in a murine model. J Biol Chem. 2011 Jan 07; 286(1):726-36. PMID: 20956523; PMCID: PMC3013031.
  15. Edwards MM, Mammadova-Bach E, Alpy F, Klein A, Hicks WL, Roux M, Simon-Assmann P, Smith RS, Orend G, Wu J, Peachey NS, Naggert JK, Lefebvre O, Nishina PM. Mutations in Lama1 disrupt retinal vascular development and inner limiting membrane formation. J Biol Chem. 2010 Mar 05; 285(10):7697-711. PMID: 20048158; PMCID: PMC2844215.
  16. Won J, Gifford E, Smith RS, Yi H, Ferreira PA, Hicks WL, Li T, Naggert JK, Nishina PM. RPGRIP1 is essential for normal rod photoreceptor outer segment elaboration and morphogenesis. Hum Mol Genet. 2009 Nov 15; 18(22):4329-39. PMID: 19679561; PMCID: PMC2766293.
  17. Near RI, Smith RS, Toselli PA, Freddo TF, Bloom AB, Vanden Borre P, Seldin DC, Lerner A. Loss of AND-34/BCAR3 expression in mice results in rupture of the adult lens. Mol Vis. 2009; 15:685-99. PMID: 19365570; PMCID: PMC2666772.
  18. Gray MP, Smith RS, Soules KA, John SW, Link BA. The aqueous humor outflow pathway of zebrafish. Invest Ophthalmol Vis Sci. 2009 Apr; 50(4):1515-21. PMID: 19060283; PMCID: PMC3642099.
  19. Chang B, Mandal MN, Chavali VR, Hawes NL, Khan NW, Hurd RE, Smith RS, Davisson ML, Kopplin L, Klein BE, Klein R, Iyengar SK, Heckenlively JR, Ayyagari R. Age-related retinal degeneration (arrd2) in a novel mouse model due to a nonsense mutation in the Mdm1 gene. Hum Mol Genet. 2008 Dec 15; 17(24):3929-41. PMID: 18805803; PMCID: PMC2638579.
  20. Maddox DM, Vessey KA, Yarbrough GL, Invergo BM, Cantrell DR, Inayat S, Balannik V, Hicks WL, Hawes NL, Byers S, Smith RS, Hurd R, Howell D, Gregg RG, Chang B, Naggert JK, Troy JB, Pinto LH, Nishina PM, McCall MA. Allelic variance between GRM6 mutants, Grm6nob3 and Grm6nob4 results in differences in retinal ganglion cell visual responses. J Physiol. 2008 Sep 15; 586(18):4409-24. PMID: 18687716; PMCID: PMC2614010.
  21. Verdoni AM, Smith RS, Ikeda A, Ikeda S. Defects in actin dynamics lead to an autoinflammatory condition through the upregulation of CXCL5. PLoS One. 2008 Jul 16; 3(7):e2701. PMID: 18628996; PMCID: PMC2442876.
  22. Won J, Smith RS, Peachey NS, Wu J, Hicks WL, Naggert JK, Nishina PM. Membrane frizzled-related protein is necessary for the normal development and maintenance of photoreceptor outer segments. Vis Neurosci. 2008 Jul-Aug; 25(4):563-74. PMID: 18764959; PMCID: PMC2727655.
  23. Howell GR, Libby RT, Jakobs TC, Smith RS, Phalan FC, Barter JW, Barbay JM, Marchant JK, Mahesh N, Porciatti V, Whitmore AV, Masland RH, John SW. Axons of retinal ganglion cells are insulted in the optic nerve early in DBA/2J glaucoma. J Cell Biol. 2007 Dec 31; 179(7):1523-37. PMID: 18158332; PMCID: PMC2373494.
  24. Libby RT, Howell GR, Pang IH, Savinova OV, Mehalow AK, Barter JW, Smith RS, Clark AF, John SW. Inducible nitric oxide synthase, Nos2, does not mediate optic neuropathy and retinopathy in the DBA/2J glaucoma model. BMC Neurosci. 2007 Dec 19; 8:108. PMID: 18093296; PMCID: PMC2211487.
  25. Brooks BP, Larson DM, Chan CC, Kjellstrom S, Smith RS, Crawford MA, Lamoreux L, Huizing M, Hess R, Jiao X, Hejtmancik JF, Maminishkis A, John SW, Bush R, Pavan WJ. Analysis of ocular hypopigmentation in Rab38cht/cht mice. Invest Ophthalmol Vis Sci. 2007 Sep; 48(9):3905-13. PMID: 17724166; PMCID: PMC1989767.
  26. Howell GR, Libby RT, Marchant JK, Wilson LA, Cosma IM, Smith RS, Anderson MG, John SW. Absence of glaucoma in DBA/2J mice homozygous for wild-type versions of Gpnmb and Tyrp1. BMC Genet. 2007 Jul 03; 8:45. PMID: 17608931; PMCID: PMC1937007.
  27. Gould DB, Marchant JK, Savinova OV, Smith RS, John SW. Col4a1 mutation causes endoplasmic reticulum stress and genetically modifiable ocular dysgenesis. Hum Mol Genet. 2007 Apr 01; 16(7):798-807. PMID: 17317786.
    View in: PubMed
  28. Haider NB, Demarco P, Nystuen AM, Huang X, Smith RS, McCall MA, Naggert JK, Nishina PM. The transcription factor Nr2e3 functions in retinal progenitors to suppress cone cell generation. Vis Neurosci. 2006 Nov-Dec; 23(6):917-29. PMID: 17266784.
    View in: PubMed
  29. Gould DB, Reedy M, Wilson LA, Smith RS, Johnson RL, John SW. Mutant myocilin nonsecretion in vivo is not sufficient to cause glaucoma. Mol Cell Biol. 2006 Nov; 26(22):8427-36. PMID: 16954374; PMCID: PMC1636791.
  30. Anderson MG, Libby RT, Mao M, Cosma IM, Wilson LA, Smith RS, John SW. Genetic context determines susceptibility to intraocular pressure elevation in a mouse pigmentary glaucoma. BMC Biol. 2006 Jul 07; 4:20. PMID: 16827931; PMCID: PMC1543659.
  31. Gwynn B, Smith RS, Rowe LB, Taylor BA, Peters LL. A mouse TRAPP-related protein is involved in pigmentation. Genomics. 2006 Aug; 88(2):196-203. PMID: 16697553.
    View in: PubMed
  32. Lyons BL, Smith RS, Hurd RE, Hawes NL, Burzenski LM, Nusinowitz S, Hasham MG, Chang B, Shultz LD. Deficiency of SHP-1 protein-tyrosine phosphatase in "viable motheaten" mice results in retinal degeneration. Invest Ophthalmol Vis Sci. 2006 Mar; 47(3):1201-9. PMID: 16505059.
    View in: PubMed
  33. Lee Y, Kameya S, Cox GA, Hsu J, Hicks W, Maddatu TP, Smith RS, Naggert JK, Peachey NS, Nishina PM. Ocular abnormalities in Large(myd) and Large(vls) mice, spontaneous models for muscle, eye, and brain diseases. Mol Cell Neurosci. 2005 Oct; 30(2):160-72. PMID: 16111892.
    View in: PubMed
  34. Libby RT, Anderson MG, Pang IH, Robinson ZH, Savinova OV, Cosma IM, Snow A, Wilson LA, Smith RS, Clark AF, John SW. Inherited glaucoma in DBA/2J mice: pertinent disease features for studying the neurodegeneration. Vis Neurosci. 2005 Sep-Oct; 22(5):637-48. PMID: 16332275.
    View in: PubMed
  35. Libby RT, Li Y, Savinova OV, Barter J, Smith RS, Nickells RW, John SW. Susceptibility to neurodegeneration in a glaucoma is modified by Bax gene dosage. PLoS Genet. 2005 Jul; 1(1):17-26. PMID: 16103918; PMCID: PMC1183523.
  36. Gould DB, Phalan FC, Breedveld GJ, van Mil SE, Smith RS, Schimenti JC, Aguglia U, van der Knaap MS, Heutink P, John SW. Mutations in Col4a1 cause perinatal cerebral hemorrhage and porencephaly. Science. 2005 May 20; 308(5725):1167-71. PMID: 15905400.
    View in: PubMed
  37. Cursiefen C, Ikeda S, Nishina PM, Smith RS, Ikeda A, Jackson D, Mo JS, Chen L, Dana MR, Pytowski B, Kruse FE, Streilein JW. Spontaneous corneal hem- and lymphangiogenesis in mice with destrin-mutation depend on VEGFR3 signaling. Am J Pathol. 2005 May; 166(5):1367-77. PMID: 15855638; PMCID: PMC1606392.
  38. Anderson MG, Libby RT, Gould DB, Smith RS, John SW. High-dose radiation with bone marrow transfer prevents neurodegeneration in an inherited glaucoma. Proc Natl Acad Sci U S A. 2005 Mar 22; 102(12):4566-71. PMID: 15758074; PMCID: PMC555465.
  39. Link BA, Gray MP, Smith RS, John SW. Intraocular pressure in zebrafish: comparison of inbred strains and identification of a reduced melanin mutant with raised IOP. Invest Ophthalmol Vis Sci. 2004 Dec; 45(12):4415-22. PMID: 15557450.
    View in: PubMed
  40. Gould DB, Miceli-Libby L, Savinova OV, Torrado M, Tomarev SI, Smith RS, John SW. Genetically increasing Myoc expression supports a necessary pathologic role of abnormal proteins in glaucoma. Mol Cell Biol. 2004 Oct; 24(20):9019-25. PMID: 15456875; PMCID: PMC517885.
  41. Gould DB, Smith RS, John SW. Anterior segment development relevant to glaucoma. Int J Dev Biol. 2004; 48(8-9):1015-29. PMID: 15558492.
    View in: PubMed
  42. Mehalow AK, Kameya S, Smith RS, Hawes NL, Denegre JM, Young JA, Bechtold L, Haider NB, Tepass U, Heckenlively JR, Chang B, Naggert JK, Nishina PM. CRB1 is essential for external limiting membrane integrity and photoreceptor morphogenesis in the mammalian retina. Hum Mol Genet. 2003 Sep 01; 12(17):2179-89. PMID: 12915475.
    View in: PubMed
  43. Mo JS, Anderson MG, Gregory M, Smith RS, Savinova OV, Serreze DV, Ksander BR, Streilein JW, John SW. By altering ocular immune privilege, bone marrow-derived cells pathogenically contribute to DBA/2J pigmentary glaucoma. J Exp Med. 2003 May 19; 197(10):1335-44. PMID: 12756269; PMCID: PMC2193785.
  44. Ikeda S, Cunningham LA, Boggess D, Hawes N, Hobson CD, Sundberg JP, Naggert JK, Smith RS, Nishina PM. Aberrant actin cytoskeleton leads to accelerated proliferation of corneal epithelial cells in mice deficient for destrin (actin depolymerizing factor). Hum Mol Genet. 2003 May 01; 12(9):1029-37. PMID: 12700171.
    View in: PubMed
  45. Libby RT, Smith RS, Savinova OV, Zabaleta A, Martin JE, Gonzalez FJ, John SW. Modification of ocular defects in mouse developmental glaucoma models by tyrosinase. Science. 2003 Mar 07; 299(5612):1578-81. PMID: 12624268.
    View in: PubMed
  46. Smith RS. Comment on 'characterization of a mutation in the lens-specific MP70 encoding gene of the mouse leading to a dominant cataract' by J.C. Graw et al., Experimental Eye Research, Vol. 73 (2001) pp 867-876. Exp Eye Res. 2002 Aug; 75(2):237; author reply 239. PMID: 12137770.
    View in: PubMed
  47. Smith RS, Korb D, John SW. A goniolens for clinical monitoring of the mouse iridocorneal angle and optic nerve. Mol Vis. 2002 Feb 25; 8:26-31. PMID: 11889463.
    View in: PubMed
  48. Smith RS, Miller JV, Sundberg JP. Intraocular teratoma in a mouse. Comp Med. 2002 Feb; 52(1):68-72. PMID: 11900416.
    View in: PubMed
  49. Anderson MG, Smith RS, Hawes NL, Zabaleta A, Chang B, Wiggs JL, John SW. Mutations in genes encoding melanosomal proteins cause pigmentary glaucoma in DBA/2J mice. Nat Genet. 2002 Jan; 30(1):81-5. PMID: 11743578.
    View in: PubMed
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