Jonathan Lin

Title(s)Professor In Residence, Pathology
SchoolHealth Sciences
vCardDownload vCard

    Collapse Research 
    Collapse Research Activities and Funding
    Molecular Pathology of Achromatopsia
    NIH/NEI R01EY027335Sep 30, 2017 - Jun 30, 2020
    Role: Principal Investigator
    Endoplasmic Reticulum Stress in Neurodegeneration
    VA I01RX002340Feb 1, 2017 - Jan 31, 2021
    Role: Principal Investigator
    Protein Homeostasis and Proteotoxicity Mechanisms
    NIH/NINDS R01NS088485Feb 1, 2015 - Jan 31, 2020
    Role: Principal Investigator
    Cellular and Molecular Mechanisms of Age-Related Retinal Degeneration
    VA I01BX002284Apr 1, 2014 - Mar 31, 2023
    Role: Principal Investigator
    Endoplasmic Reticulum Stress in Retinal Degeneration
    NIH/NEI R01EY020846Sep 1, 2010 - May 31, 2015
    Role: Principal Investigator
    Endoplasmic Reticulum Stress in Retinal Degeneration
    NIH/NEI K08EY018313Aug 1, 2007 - Jul 31, 2012
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
    List All   |   Timeline
    1. Lin JH, Brunson A, Romano PS, Mell MW, Humphries MD. Endovascular-First Treatment Is Associated With Improved Amputation-Free Survival in Patients With Critical Limb Ischemia. Circ Cardiovasc Qual Outcomes. 2019 Aug; 12(8):e005273. PMID: 31357888.
      View in: PubMed
    2. Mastey RR, Georgiou M, Langlo CS, Kalitzeos A, Patterson EJ, Kane T, Singh N, Vincent A, Moore AT, Tsang SH, Lin JH, Young MP, Hartnett ME, Héon E, Kohl S, Michaelides M, Carroll J. Characterization of Retinal Structure in ATF6-Associated Achromatopsia. Invest Ophthalmol Vis Sci. 2019 Jun 03; 60(7):2631-2640. PMID: 31237654.
      View in: PubMed
    3. Yang P, Toomey C, Lin J, Kikkawa DO, Korn BS, Harrison A. Beware of the sneeze. Surv Ophthalmol. 2019 Apr 10. PMID: 30978335.
      View in: PubMed
    4. Men CJ, Wu F, Lee BW, Lin JH, Korn BS, Kikkawa DO. Odontogenic choristoma presenting as dermolipoma. Orbit. 2019 Jan 10; 1-3. PMID: 30628515.
      View in: PubMed
    5. Toomey CB, Fraser K, Thorson JA, Goldbaum MH, Lin JH. GNAQ and PMS1 Mutations Associated with Uveal Melanoma, Ocular Surface Melanosis, and Nevus of Ota. Ocul Oncol Pathol. 2019 Jun; 5(4):267-272. PMID: 31367589.
      View in: PubMed
    6. Orrù CD, Soldau K, Cordano C, Llibre-Guerra J, Green AJ, Sanchez H, Groveman BR, Edland SD, Safar JG, Lin JH, Caughey B, Geschwind MD, Sigurdson CJ. Prion Seeds Distribute throughout the Eyes of Sporadic Creutzfeldt-Jakob Disease Patients. MBio. 2018 11 20; 9(6). PMID: 30459197.
      View in: PubMed
    7. Yuan SH, Hiramatsu N, Liu Q, Sun XV, Lenh D, Chan P, Chiang K, Koo EH, Kao AW, Litvan I, Lin JH. Tauopathy-associated PERK alleles are functional hypomorphs that increase neuronal vulnerability to ER stress. Hum Mol Genet. 2018 11 15; 27(22):3951-3963. PMID: 30137327.
      View in: PubMed
    8. Choi MY, Kato S, Wang HY, Lin JH, Lanman RB, Kurzrock R. JAK2 V617F mutation in plasma cell-free DNA preceding clinically overt myelofibrosis: Implications for early diagnosis. Cancer Biol Ther. 2018 Aug 03; 19(8):664-668. PMID: 29565699.
      View in: PubMed
    9. Kroeger H, Chiang WC, Felden J, Nguyen A, Lin JH. ER stress and unfolded protein response in ocular health and disease. FEBS J. 2019 Jan; 286(2):399-412. PMID: 29802807.
      View in: PubMed
    10. Kroeger H, Grimsey N, Paxman R, Chiang WC, Plate L, Jones Y, Shaw PX, Trejo J, Tsang SH, Powers E, Kelly JW, Wiseman RL, Lin JH. The unfolded protein response regulator ATF6 promotes mesodermal differentiation. Sci Signal. 2018 Feb 13; 11(517). PMID: 29440509.
      View in: PubMed
    11. Alameddine RM, Ko AC, Mimura M, Parker BA, Lin JH, Korn BS, Kikkawa DO. Orbital fat regeneration following hormonal treatment of metastatic breast carcinoma. Orbit. 2018 Jun; 37(3):187-190. PMID: 29072521.
      View in: PubMed
    12. Skorczyk-Werner A, Chiang WC, Wawrocka A, Wicher K, Jarmuz-Szymczak M, Kostrzewska-Poczekaj M, Jamsheer A, Ploski R, Rydzanicz M, Pojda-Wilczek D, Weisschuh N, Wissinger B, Kohl S, Lin JH, Krawczynski MR. Autosomal recessive cone-rod dystrophy can be caused by mutations in the ATF6 gene. Eur J Hum Genet. 2017 11; 25(11):1210-1216. PMID: 28812650.
      View in: PubMed
    13. Rodvold JJ, Chiu KT, Hiramatsu N, Nussbacher JK, Galimberti V, Mahadevan NR, Willert K, Lin JH, Zanetti M. Intercellular transmission of the unfolded protein response promotes survival and drug resistance in cancer cells. Sci Signal. 2017 Jun 06; 10(482). PMID: 28588081.
      View in: PubMed
    14. Whipple KM, Godfrey KJ, Solomon JP, Lin JH, Korn BS, Kikkawa DO. Glomuvenous Malformation: A Rare Periorbital Lesion of the Thermoregulatory Apparatus. Ophthalmic Plast Reconstr Surg. 2017 Mar/Apr; 33(2):e36-e37. PMID: 27065433.
      View in: PubMed
    15. Biswas P, Duncan JL, Maranhao B, Kozak I, Branham K, Gabriel L, Lin JH, Barteselli G, Navani M, Suk J, Parke M, Schlechter C, Weleber RG, Heckenlively JR, Dagnelie G, Lee P, Riazuddin SA, Ayyagari R. Genetic analysis of 10 pedigrees with inherited retinal degeneration by exome sequencing and phenotype-genotype association. Physiol Genomics. 2017 Apr 01; 49(4):216-229. PMID: 28130426.
      View in: PubMed
    16. Chiang WC, Chan P, Wissinger B, Vincent A, Skorczyk-Werner A, Krawczynski MR, Kaufman RJ, Tsang SH, Héon E, Kohl S, Lin JH. Achromatopsia mutations target sequential steps of ATF6 activation. Proc Natl Acad Sci U S A. 2017 01 10; 114(2):400-405. PMID: 28028229.
      View in: PubMed
    17. Kinori M, Ko AC, Alameddine RM, Snyder V, Lin JH, Korn BS, Granet DB. Extraocular Muscle Fibrosis in Idiopathic Orbital Inflammation. J Pediatr Ophthalmol Strabismus. 2016 Jul 01; 53(4):256. PMID: 27428626.
      View in: PubMed
    18. Kim JS, Scawn RL, Lee BW, Lin JH, Korn BS, Kikkawa DO. Masquerading Orbital Sarcoidosis with Isolated Extraocular Muscle Involvement. Open Ophthalmol J. 2016; 10:140-145. PMID: 28484581.
      View in: PubMed
    19. Chan P, Stolz J, Kohl S, Chiang WC, Lin JH. Endoplasmic reticulum stress in human photoreceptor diseases. Brain Res. 2016 10 01; 1648(Pt B):538-541. PMID: 27117871.
      View in: PubMed
    20. Westenskow PD, Bucher F, Bravo S, Kurihara T, Feitelberg D, Paris LP, Aguilar E, Lin JH, Friedlander M. iPSC-Derived Retinal Pigment Epithelium Allografts Do Not Elicit Detrimental Effects in Rats: A Follow-Up Study. Stem Cells Int. 2016; 2016:8470263. PMID: 26880994; PMCID: PMC4736415.
    21. Chiang WC, Joseph V, Yasumura D, Matthes MT, Lewin AS, Gorbatyuk MS, Ahern K, LaVail MM, Lin JH. Ablation of Chop Transiently Enhances Photoreceptor Survival but Does Not Prevent Retinal Degeneration in Transgenic Mice Expressing Human P23H Rhodopsin. Adv Exp Med Biol. 2016; 854:185-91. PMID: 26427410; PMCID: PMC4731204.
    22. Alavi MV, Chiang WC, Kroeger H, Yasumura D, Matthes MT, Iwawaki T, LaVail MM, Gould DB, Lin JH. In Vivo Visualization of Endoplasmic Reticulum Stress in the Retina Using the ERAI Reporter Mouse. Invest Ophthalmol Vis Sci. 2015 Oct; 56(11):6961-70. PMID: 26513501; PMCID: PMC4627472.
    23. Kobalka PJ, Abboud JP, Liao X, Jones K, Lee BW, Korn BS, Kikkawa DO, Lin JH. p16INK4A expression is frequently increased in periorbital and ocular squamous lesions. Diagn Pathol. 2015 Sep 24; 10:175. PMID: 26400483; PMCID: PMC4581440.
    24. Lee BW, Kumar UR, Lin JH, Amaro DE, Kikkawa DO, Alameddine RM, Lowe MC, Hilger PA, Vinetz JM, Korn BS. Cysticercosis with an Orbital Tropism in Twins. Am J Trop Med Hyg. 2015 Oct; 93(4):828-30. PMID: 26217041; PMCID: PMC4596607 [Available on 10/07/16].
    25. Kohl S, Zobor D, Chiang WC, Weisschuh N, Staller J, Gonzalez Menendez I, Chang S, Beck SC, Garcia Garrido M, Sothilingam V, Seeliger MW, Stanzial F, Benedicenti F, Inzana F, Héon E, Vincent A, Beis J, Strom TM, Rudolph G, Roosing S, Hollander AI, Cremers FP, Lopez I, Ren H, Moore AT, Webster AR, Michaelides M, Koenekoop RK, Zrenner E, Kaufman RJ, Tsang SH, Wissinger B, Lin JH. Mutations in the unfolded protein response regulator ATF6 cause the cone dysfunction disorder achromatopsia. Nat Genet. 2015 Jul; 47(7):757-65. PMID: 26029869; PMCID: PMC4610820.
    26. Hiramatsu N, Chiang WC, Kurt TD, Sigurdson CJ, Lin JH. Multiple Mechanisms of Unfolded Protein Response-Induced Cell Death. Am J Pathol. 2015 Jul; 185(7):1800-8. PMID: 25956028; PMCID: PMC4484218.
    27. Zou AE, Ku J, Honda TK, Yu V, Kuo SZ, Zheng H, Xuan Y, Saad MA, Hinton A, Brumund KT, Lin JH, Wang-Rodriguez J, Ongkeko WM. Transcriptome sequencing uncovers novel long noncoding and small nucleolar RNAs dysregulated in head and neck squamous cell carcinoma. RNA. 2015 Jun; 21(6):1122-34. PMID: 25904139; PMCID: PMC4436665.
    28. Karam R, Lou CH, Kroeger H, Huang L, Lin JH, Wilkinson MF. The unfolded protein response is shaped by the NMD pathway. EMBO Rep. 2015 May; 16(5):599-609. PMID: 25807986; PMCID: PMC4428047.
    29. Salganik M, Sergeyev VG, Shinde V, Meyers CA, Gorbatyuk MS, Lin JH, Zolotukhin S, Gorbatyuk OS. The loss of glucose-regulated protein 78 (GRP78) during normal aging or from siRNA knockdown augments human alpha-synuclein (a-syn) toxicity to rat nigral neurons. Neurobiol Aging. 2015 Jun; 36(6):2213-23. PMID: 25863526; PMCID: PMC4433578.
    30. Chiang WC, Kroeger H, Sakami S, Messah C, Yasumura D, Matthes MT, Coppinger JA, Palczewski K, LaVail MM, Lin JH. Robust Endoplasmic Reticulum-Associated Degradation of Rhodopsin Precedes Retinal Degeneration. Mol Neurobiol. 2015 Aug; 52(1):679-95. PMID: 25270370; PMCID: PMC4383737.
    31. Muller K, Lin JH. Orbital granulomatosis with polyangiitis (Wegener granulomatosis): clinical and pathologic findings. Arch Pathol Lab Med. 2014 Aug; 138(8):1110-4. PMID: 25076302; PMCID: PMC4140401.
    32. Lin J, Jain S, Sun X, Liu V, Sato YZ, Jimenez-Fernandez S, Newfield RS, Pourfarzib R, Tremoulet AH, Gordon JB, Daniels LB, Burns JC. Lipoprotein particle concentrations in children and adults following Kawasaki disease. J Pediatr. 2014 Oct; 165(4):727-31. PMID: 25039043; PMCID: PMC4207833.
    33. Ouyang H, Xue Y, Lin Y, Zhang X, Xi L, Patel S, Cai H, Luo J, Zhang M, Zhang M, Yang Y, Li G, Li H, Jiang W, Yeh E, Lin J, Pei M, Zhu J, Cao G, Zhang L, Yu B, Chen S, Fu XD, Liu Y, Zhang K. WNT7A and PAX6 define corneal epithelium homeostasis and pathogenesis. Nature. 2014 Jul 17; 511(7509):358-61. PMID: 25030175; PMCID: PMC4610745.
    34. Wert KJ, Lin JH, Tsang SH. General pathophysiology in retinal degeneration. Dev Ophthalmol. 2014; 53:33-43. PMID: 24732759; PMCID: PMC4405532.
    35. Hiramatsu N, Messah C, Han J, LaVail MM, Kaufman RJ, Lin JH. Translational and posttranslational regulation of XIAP by eIF2a and ATF4 promotes ER stress-induced cell death during the unfolded protein response. Mol Biol Cell. 2014 May; 25(9):1411-20. PMID: 24623724; PMCID: PMC4004591.
    36. Jerry Chiang WC, Lin JH. The effects of IRE1, ATF6, and PERK signaling on adRP-linked rhodopsins. Adv Exp Med Biol. 2014; 801:661-7. PMID: 24664756.
      View in: PubMed
    37. Kroeger H, LaVail MM, Lin JH. Endoplasmic reticulum stress in vertebrate mutant rhodopsin models of retinal degeneration. Adv Exp Med Biol. 2014; 801:585-92. PMID: 24664747; PMCID: PMC4103620.
    38. Joshi-Barr S, Bett C, Chiang WC, Trejo M, Goebel HH, Sikorska B, Liberski P, Raeber A, Lin JH, Masliah E, Sigurdson CJ. De novo prion aggregates trigger autophagy in skeletal muscle. J Virol. 2014 Feb; 88(4):2071-82. PMID: 24307586; PMCID: PMC3911572.
    39. Hodgson N, Whipple K, Lin JH, Brumund KT, Kikkawa DO, Korn BS. Bilateral squamous cell carcinoma of the lacrimal sac. Ophthalmic Plast Reconstr Surg. 2013 Nov-Dec; 29(6):e149-51. PMID: 24217491.
      View in: PubMed
    40. Mahajan VB, Lin JH. Lymphocyte infiltration in CAPN5 autosomal dominant neovascular inflammatory vitreoretinopathy. Clin Ophthalmol. 2013; 7:1339-45. PMID: 23861576.
      View in: PubMed
    41. Kroeger H, Messah C, Ahern K, Gee J, Joseph V, Matthes MT, Yasumura D, Gorbatyuk MS, Chiang WC, LaVail MM, Lin JH. Induction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degeneration. Invest Ophthalmol Vis Sci. 2012 Nov 09; 53(12):7590-9. PMID: 23074209; PMCID: PMC3495601.
    42. Chiang WC, Hiramatsu N, Messah C, Kroeger H, Lin JH. Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulation. Invest Ophthalmol Vis Sci. 2012 Oct 01; 53(11):7159-66. PMID: 22956602; PMCID: PMC3474590.
    43. Gorbatyuk MS, Shabashvili A, Chen W, Meyers C, Sullivan LF, Salganik M, Lin JH, Lewin AS, Muzyczka N, Gorbatyuk OS. Glucose regulated protein 78 diminishes a-synuclein neurotoxicity in a rat model of Parkinson disease. Mol Ther. 2012 Jul; 20(7):1327-37. PMID: 22434142; PMCID: PMC3392977.
    44. Shinde VM, Sizova OS, Lin JH, LaVail MM, Gorbatyuk MS. ER stress in retinal degeneration in S334ter Rho rats. PLoS One. 2012; 7(3):e33266. PMID: 22432009; PMCID: PMC3303830.
    45. Chiang WC, Messah C, Lin JH. IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsin. Mol Biol Cell. 2012 Mar; 23(5):758-70. PMID: 22219383; PMCID: PMC3290636.
    46. Kroeger H, Chiang WC, Lin JH. Endoplasmic reticulum-associated degradation (ERAD) of misfolded glycoproteins and mutant P23H rhodopsin in photoreceptor cells. Adv Exp Med Biol. 2012; 723:559-65. PMID: 22183378; PMCID: PMC3651033.
    47. Gorbatyuk MS, Gorbatyuk OS, LaVail MM, Lin JH, Hauswirth WW, Lewin AS. Functional rescue of P23H rhodopsin photoreceptors by gene delivery. Adv Exp Med Biol. 2012; 723:191-7. PMID: 22183333; PMCID: PMC3365920.
    48. Mantuano E, Henry K, Yamauchi T, Hiramatsu N, Yamauchi K, Orita S, Takahashi K, Lin JH, Gonias SL, Campana WM. The unfolded protein response is a major mechanism by which LRP1 regulates Schwann cell survival after injury. J Neurosci. 2011 Sep 21; 31(38):13376-85. PMID: 21940431; PMCID: PMC3188465.
    49. Vasireddy V, Chavali VR, Joseph VT, Kadam R, Lin JH, Jamison JA, Kompella UB, Reddy GB, Ayyagari R. Rescue of photoreceptor degeneration by curcumin in transgenic rats with P23H rhodopsin mutation. PLoS One. 2011; 6(6):e21193. PMID: 21738619; PMCID: PMC3126808.
    50. Hiramatsu N, Joseph VT, Lin JH. Monitoring and manipulating mammalian unfolded protein response. Methods Enzymol. 2011; 491:183-98. PMID: 21329801; PMCID: PMC3658618.
    51. Mahajan VB, Vallone JG, Lin JH, Mullins RF, Ko AC, Folk JC, Stone EM. T-cell infiltration in autosomal dominant neovascular inflammatory vitreoretinopathy. Mol Vis. 2010 Jun 08; 16:1034-40. PMID: 20596252; PMCID: PMC2893052.
    52. Gorbatyuk MS, Knox T, LaVail MM, Gorbatyuk OS, Noorwez SM, Hauswirth WW, Lin JH, Muzyczka N, Lewin AS. Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78. Proc Natl Acad Sci U S A. 2010 Mar 30; 107(13):5961-6. PMID: 20231467.
      View in: PubMed
    53. Lin JH, Lavail MM. Misfolded proteins and retinal dystrophies. Adv Exp Med Biol. 2010; 664:115-21. PMID: 20238009; PMCID: PMC2955894.
    54. Hollien J, Lin JH, Li H, Stevens N, Walter P, Weissman JS. Regulated Ire1-dependent decay of messenger RNAs in mammalian cells. J Cell Biol. 2009 Aug 10; 186(3):323-31. PMID: 19651891; PMCID: PMC2728407.
    55. Lisbona F, Rojas-Rivera D, Thielen P, Zamorano S, Todd D, Martinon F, Glavic A, Kress C, Lin JH, Walter P, Reed JC, Glimcher LH, Hetz C. BAX inhibitor-1 is a negative regulator of the ER stress sensor IRE1alpha. Mol Cell. 2009 Mar 27; 33(6):679-91. PMID: 19328063; PMCID: PMC2818874.
    56. Lin JH, Li H, Zhang Y, Ron D, Walter P. Divergent effects of PERK and IRE1 signaling on cell viability. PLoS One. 2009; 4(1):e4170. PMID: 19137072; PMCID: PMC2614882.
    57. Lin JH, Walter P, Yen TS. Endoplasmic reticulum stress in disease pathogenesis. Annu Rev Pathol. 2008; 3:399-425. PMID: 18039139; PMCID: PMC3653419.
    58. Lin JH, Li H, Yasumura D, Cohen HR, Zhang C, Panning B, Shokat KM, Lavail MM, Walter P. IRE1 signaling affects cell fate during the unfolded protein response. Science. 2007 Nov 09; 318(5852):944-9. PMID: 17991856.
      View in: PubMed