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From 1950-2001, there were a total of 104 peer review publications on Progeria – an average of about two per year. From 2002-2005, 52 publications appeared- that's a 550% annual increase!

Is there any other disease field in the history of science that has been able to generate this kind of interest and progress so quickly? We all have great hopes that this will translate into treatments and cure, the mission of PRF, in the near future.

  1. Abdenur JE, Brown WT, Freidman S, Smith M, Zdanowicz M, Lifshitz F. Endogenous growth hormone resistance and malnutrition in children with Hutchinson-Gilford progeria syndrome (HGP). Pediatr. Res. 1991;29:73A (abstr). (no abstract available online)
  2. Abdenur JE, Brown WT, Friedman S, Smith M, Lifshitz F. Response to nutritional and growth hormone treatment in progeria. Metabolism. 1997;46(8):851 - 856.
  3. Allsopp RC, Vaziri H, Patterson C, Goldstein S, Younglai EV, Futcher AB, Greider CW, Harley CB.
    Telomere length predicts replicative capacity of human fibroblasts. Proc Natl Acad Sci U S A. 1992 Nov 1;89(21):10114-8. PMID: 1438199 [PubMed - indexed for MEDLINE]
  4. Ayres SC, Mihan R. Progeria: a possible therapeutic approach. JAMA. 1974;227:1381. (no abstract available online)
  5. Badame AJ. Progeria. Archives of Dermatology. 1989;125:540 - 544.
  6. Baker PB, Baba N, Boesel CP. Cardiovascular abnormalities in progeria. Arch Pathol Lab Med. 1981;105:384 - 386.
  7. Batstone MD, Macleod AW.
    Related Articles, Links
    Oral and maxillofacial surgical considerations for a case of Hutchinson-Gilford progeria.
    Int J Paediatr Dent. 2002 Nov;12(6):429-32.
    PMID: 12452985 [PubMed - indexed for MEDLINE]
  8. Biswas A, Reddy A. Images in clinical practice. Progeria. Indian-Pediatr. 1997;34(10):945 -946. (no abstract available online)
  9. Brown WT, J. Epstein, J.B. Little. Progeria cells are stimulated to repair DNA by cocultivation with normal cells. Experimental Cell Research. 1976;97:291-296. (no abstract available online)
  10. Brown WT, J.B. Little, J.Epstein, J.R. Williams. DNA repair defect in progeric cells. Birth Defects. 1978;14:417-430. (no abstract available online)  
  11. Brown WT. Human mutations affecting aging - A review. Mechanisms of Aging and Development. 1979;9:325-336.
  12. Brown WT, Darlington GJ. Thermolabile enzymes in progeria and werner syndrome: Evidence contrary to the protein error hypothesis. American Journal of Human Genetics. 1980;32:614-619.
  13. Brown WT, Darlingtron GJ, Arnold A, Fotino M. Detection of HLA antigens on progeria syndrome fibroblasts. Clinical Genetics. 1980;17:213-219.1980;32:614-619.
  14. Brown WT, J. Ford, E.L. Gershey. Variation of DNA repair in progeria cells unrelated to growth conditions. Biochemical and Biophysical Research Communications. 1981;97:347-353. (no abstract available online)
  15. Brown WT, F.J. Kieras, G.E. Houck, R. Dutkowski, E.C. Jenkins. A comparison of adult and childhood progerias: Werner syndrome and Hutchinson-Gilford Progeria Syndrome In: Werner's Syndrome and Human Aging. Advances in Experimental Medicine and Biology. 1985;190:229-244. (no abstract available online)
  16. Brown WT, Zebrower M, Kieras FJ. Progeria, A model disease for the study of accelerated aging. In: Woodhead AD, Blackett AD, Hollaender A, eds. Molecular Biology of Aging: Plenum Publishing Corporation; 1985:375 - 396. (no abstract available online)
  17. Brown WT. Genetics of human aging and premature aging syndromes: relationship to atherogenesis. In: S.R. Bates ECG, ed. Atherogenesis and Aging. New York: Springer Veriag; 1987:177-182. (no abstract available online)
  18. Brown WT. Human genetic models for aging research. In: B.Kent RB, ed.Human Aging Research; Concepts and Techniques. Vol. 34. New York: Raven Press; 1988:163-181. (no abstract available online)
  19. Brown WT. Genetic.shtmlects of human aging. In: Lesnoff-Caraveglia G, ed. Aging in a Technological Society. New York: Human Sciences Press Inc.; 1988:184-203. (no abstract available online)
  20. Brown WT. Contribution of in vitro skin fibroblast studies from individuals with genetic disease that predispose to accelerated aging phenomena to our understanding of the aging process. In: Balin A, Kligman A., ed. Aging and the Skin. New York: Raven Press; 1988:93-120. (no abstract available online)
  21. Brown WT. Genetic diseases of premature aging as models of senescence. Annu. Rev. Gerontol. Geriatr. 1990;10:23-42. (no abstract available online)
  22. Brown WT, Zebrower M, Kieras FJ. Progeria: A genetic disease model of premature aging. In: Harrison D, ed. Genetic Effects on Aging II. Caldwell, NJ: Telford Press; 1990:521-542. (no abstract available online)
  23. Brown WT. Progeria: a human-disease model of accelerated aging. American Journal of Clinical Nutrition. 1992;55:1222S-1224S.
  24. Capell BC, Erdos MR, Madigan JP, Fiordalisi JJ, Varga R, Conneely KN, Gordon LB, Der CJ, Cox AD, Collins FS.
    Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome.
    Proc Natl Acad Sci U S A. 2005 Sep 6;102(36):12879-84. Epub 2005 Aug 29.
    PMID: 16129833 [PubMed - indexed for MEDLINE]
  25. Chapin JW, Kahre J. Progeria and anesthesia. Anesth. Analg. 1979;58(5):424-425. (no abstract available online)
  26. Chen L, Lee L, Kudlow BA, Dos Santos HG, Sletvold O, Shafeghati Y, Botha EG, Garg A, Hanson NB, Martin GM, Mian IS, Kennedy BK, Oshima J.
    LMNA mutations in atypical Werner's syndrome.
    Lancet. 2003 Aug 9;362(9382):440-5.
  27. Clark MA, Weiss AS. Elevated levels of glycoprotein gp200 in progeria fibroblasts.Mol Cell Biochem. 1993;120(1):51-60.
  28. Clark MA, Weiss AS. Hutchinson-Gilford progeria types defined by differential binding of lectin DSA. Biochim. Biophys. Acta. 1995;1270(2-3):142 - 148.
  29. Colige A, Nusgen B, Lapiere CM. Altered response of progeria fibroblasts to epidermal growth factor. J cell Sci. 1991;100(Pt 3):649-655.
  30. Csoka AB, English SB, Simkevich CP, Ginzinger DG, Butte AJ, Schatten GP, Rothman FG, Sedivy JM. Genome-scale expression profiling of Hutchinson-Gilford progeria syndrome reveals widespread transcriptional misregulation leading to mesodermal/mesenchymal defects and accelerated atherosclerosis. Aging Cell. 2004; 3(4): 234.
  31. Dahl KN, Scaffidi P, Islam MF, Yodh AG, Wilson KL, Misteli T. Distinct structural and mechanical properties of the nuclear lamina in Hutchinson-Gilford progeria syndrome. Proc Natl Acad Sci U S A. 2006 Jun 26; [Epub ahead of print] PMID: 16801550 [PubMed - as supplied by publisher]. 
  32. Danes BS. Progeria: A Cell Culture Study on Aging. J. Clin. Invest. 1971;50:2000 - 2003. (no abstract available online)
  33. Darlington GJ, Dutkowski R, Brown WT. Sister chromatid exchange frequencies in Progeria and Werner syndrome patients. American Journal of Human Genetics. 1981;33:762-766.
  34. DeBusk FL. The Hutchinson-Gilford Progeria Syndrome. The Journal of Pediatrics. 1972;80(4):697 - 794. (no abstract available online)
  35. De Sandre-Giovannoli A, Bernard R, Cau P, Navarro C, Amiel J, Boccaccio I, Lyonnet S, Stewart CL, Munnich A, Le Merrer M, Levy N.
    Related Articles, Links
    Lamin A Truncation in Hutchinson-Gilford Progeria.
    Science. 2003 Apr 17
  36. Delahunt B, Stehbens WE, Gilbert-Barness E, Shozawa T, Ruger BM. Progeria kidney has abnormal mesenglial collagen distribution. Pediatr Nephrol 2000;15(3-4):279-285.
  37. Delgado Luengo W, Rojas Martinez A, Ortiz Lopez R, Martinez Basalo C, Rojas-Atencio A, Quintero M, Borjas L, Morales-Machin A, Gonzalez Ferrer S, Pineda Bernal L, Canizalez-Tarazona J, Pena J, Delgado Luengo J, Chacin Hernandez J, Chong Chang J.           Related Articles,Links
    Del(1)(q23) in a patient with Hutchinson-Gilford progeria.
    Am J Med Genet. 2002 Dec 1;113(3):298-301.
    PMID: 12439901 [PubMed - indexed for MEDLINE]
  38. de Paula Rodrigues GH, das Eiras Tamega I, Duque G, Spinola Dias Neto V.
    Related Articles,Links
    Severe bone changes in a case of Hutchinson-Gilford syndrome.
    Ann Genet. 2002 Jul-Sep;45(3):151-5.
    PMID: 12381448 [PubMed - indexed for MEDLINE
  39. Dyck JD, David TE, Burke B, Webb GD, Henderson MA, Fowler RS. Management of coronary artery disease in Hutchinson-Gilford syndrome. J Pediatr. 1987 Sep;111(3):407-10. No abstract available. PMID: 2957478 [PubMed - indexed for MEDLINE]
  40. Dyer C, Sinclair A. The premature ageing syndromes: insights into the ageing process. Age-Ageing. 1998;27(1):73 - 80.
  41. Epstein J, Williams JR, Little JB. Rate of DNA repair in progeric and normal human fibroblasts. Biochem Biophys Res Commun. 1974 Aug 5;59(3):850-7. No abstract available. PMID: 4415479 [PubMed - indexed for MEDLINE]
  42. Erdem N, Gunes AT, Avci O, Osma E. A case of Hutchinson-Gilford Progeria Syndrome mimicking scleredema in early infancy. Dermatology. 1994;188:318 - 321.
  43. Eriksson M, Brown WT, Gordon LB, Glynn MW, Singer J, Scott L, Erdos MR, Robbins CM, Moses TY, Berglund P, Dutra A, Pak E, Durkin S, Csoka AB, Boehnke M, Glover TW, Collins FS.
    Related Articles, Links
    Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome.
    Nature. 2003 Apr 25 [epub ahead of print]
    PMID: 12714972 [PubMed - as supplied by publisher]
  44. Faivre L, Van Kien PK, Madinier-Chappat N, Nivelon-Chevallier A, Beer F, LeMerrer M. Can Hutchinson-Gilford progeria syndrome be a neonatal condition? Am J Med Genet. 1999 Dec 22;87(5):450-2; discussion 453-4. No abstract available. PMID: 10594888 [PubMed - indexed for MEDLINE]
  45. Fernandez-Palazzi F, McLaren AT, DF DFS. Report on a case of Hutchinson-Gilford progeria, with special reference to orthopedic problems. Eur J Pediatr Surg. 1992;2(6):378-382.
  46. Fong LG, Frost D, Meta M, Qiao X, Yang SH, Coffinier C, Young SG.
    A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.
    Science. 2006 Mar 17;311(5767):1621-3. Epub 2006 Feb 16.
    PMID: 16484451 [PubMed - indexed for MEDLINE]
  47. Fong LG, Ng JK, Lammerding J, Vickers TA, Meta M, Cote N, Gavino B, Qiao X, Chang SY, Young SR, Yang SH, Stewart CL, Lee RT, Bennett CF, Bergo MO, Young SG. Prelamin A and lamin A appear to be dispensable in the nuclear lamina. J Clin Invest. 2006 Mar;116(3):743-52. PMID: 16511604 [PubMed - indexed for MEDLINE]
  48. Fossel M. Human ageing and progeria. J. Pediatr. Endocrinol. Metab. 2000;13 Suppl. 6:1477-1481.
  49. Franklyn PP. Progeria in siblings. Clin Radiol. 1976;27:327 - 333.
  50. Gabr M, Hashem N, Hashem M, Fahmi A, Safouh M. Progeria: a pathologic study. J. Pediatr. 1960;57:70. (no abstract available online)
  51. Gamble JG. Hip disease in Hutchinson-Gilford progeria syndrome. J. Pediatric Orthopedics. 1984;4:585-589. (no abstract available online)
  52. Gilford H. Progeria, a form of senilism. Practitioner. 1904;73:188. (no abstract available online)
  53. Gillar PJ, Kaye CI, McCourt JW. Progressive early dermatologic changes in Hutchinson-Gilford progeria syndrome. Pediatr Dermatol. 1991 Sep;8(3):199-206. PMID: 1745628 [PubMed - indexed for MEDLINE]
  54. Giro M, Davidson JM. Familial co-segregation of the elastin phenotype in skin fibroblasts from Hutchinson-Gilford progeria. Mech. Ageing Dev. 1993;70(3):163 - 36.
  55. Glynn MW, Glover TW.
    Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition.
    Hum Mol Genet. 2005 Oct 15;14(20):2959-69. Epub 2005 Aug 26.
    PMID: 16126733 [PubMed - indexed for MEDLINE]
  56. Goldman RD, Shumaker DK, Erdos MR, Eriksson M, Goldman AE, Gordon LB, Gruenbaum Y, Khuon S, Mendez M, Varga R, Collins FS.
    Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome.
    Proc Natl Acad Sci U S A. 2004 Jun 15;101(24):8963-8. Epub 2004 Jun 7.
    PMID: 15184648 [PubMed - indexed for MEDLINE]
  57. Goldstein S, Moerman EJ. Heat-labile enzymes in circulating erythrocytes of a progeria family. Am J. Hum Genet. 1978;30:167.
  58. Gordon LB, Harten IA, Patti ME, Lichtenstein AH.
    Reduced adiponectin and HDL cholesterol without elevated C-reactive protein: clues to the biology of premature atherosclerosis in Hutchinson-Gilford Progeria Syndrome.
    J Pediatr. 2005 Mar;146(3):336-41.
    PMID: 15756215 [PubMed - indexed for MEDLINE]
  59. Gordon LB, Harten IA, Calabro A, Sugumaran G, Csoka AB, Brown WT, Hascall V, Toole BP.
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    Hyaluronan is not elevated in urine or serum in Hutchinson-Gilford Progeria Syndrome.
    Hum Genet. 2003 May 1 [epub ahead of print]
    PMID: 12728312 [PubMed - as supplied by publisher]
  60. Green LN. Progeria with carotid artery aneurysms: report of a case. . Arch Neurol. 1981;38(10):659-661. (no abstract available online)
  61. Ha JW, Shim WH, Chung NS. Cardiovascular findings of Hutchinson-Gilford syndrome--a Doppler and two-dimensional echocardiographic study. Yonsei Med J. 1993;34(4):352-355.
  62. Hall JW 3rd, Denneny JC 3rd. Audiologic and otolaryngologic findings in progeria: case report. J Am Acad Audiol. 1993;4(2):116-121.
  63. Harjacek M, Batinic; D, Sarnavka V, Uzarevic B, Mardesic D, Marusic M. Immunological.shtmlects of progeria (Hutchinson-Gilford syndrome) in a 15-month-old child. Eur J Pediatr. 1990;150(1):40-2.
  64. Hasty MF, William F. Vann J. Progeria in a pediatric dental patient: literature review and case report. Pediatric Dentistry. 1988;10(4):314 - 319. (no abstract available online)
  65. Hoeffel JC, Mainard L, Chastagner P, Hoeffel CC. Mandibulo-acral dysplasia. Skeletal Radiol. 2000 Nov;29(11):668-71. PMID: 11201039 [PubMed - indexed for MEDLINE]
  66. Huang S, Chen L, Libina N, Janes J, Martin GM, Campisi J, Oshima J.
    Correction of cellular phenotypes of Hutchinson-Gilford Progeria cells by RNA interference.
    Hum Genet. 2005 Dec;118(3-4):444-50. Epub 2005 Oct 6.
    PMID: 16208517 [PubMed - indexed for MEDLINE]
  67. Inazumi T, Tajima S, Nishikawa T. Overexcretion of low-sulphated chondroitin sulphate in the urine of the patientresembling progeroid. Dermatology. 1997;195(3):296-300. PMID: 9407187 [PubMed - indexed for MEDLINE]
  68. Jang KA, Han MH, Choi JH, Sung KJ, Moon KC, Koh JK. Progeroid syndrome: association with connective tissue disease? Pediatr Dermatol. 1998 Nov-Dec;15(6):487-9. No abstract available. PMID: 9875981 [PubMed - indexed for MEDLINE]
  69. Jansen T, Romiti R. Progeria infantum (Hutchinson-Gilford syndrome) associated with scleroderma-like lesions and acro-osteolysis: a case report and brief review of the literature. Pediatr Dermatol. 2000;17(4):282-285.
  70. Keay AJ, Oliver MF, Boyd GS. Progeria and atherosclerosis. Journal unkown.1955:410-413. (no abstract available online)
  71. Kieras FJ, Brown WT, George E. Houck J, Zebrower M. Elevation of urinary hyaluronic acid in werner's syndrome and progeria. Biochemical Medicine and Metabolic Biology. 1986;36:276 - 282.
  72. Landon JF. Heredity in progeria. Archives of Pediatrics. 1954;71:163-72. (no abstract available online)
  73. Lemire JM, Patis C, Gordon LB, Sandy JD, Toole BP, Weiss AS. Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford
    Progeria Syndrome dermal fibroblasts. Mech Ageing Dev. 2006 Apr 28; [Epub
    ahead of print] PMID: 16650460 [PubMed - as supplied by publisher]
  74. Lewis M.
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    PRELP, collagen, and a theory of Hutchinson-Gilford progeria.
    Ageing Res Rev. 2003 Jan;2(1):95-105.
    PMID: 12437997 [PubMed - in process]
  75. Liu Y, Rusinol A, Sinensky M, Wang Y, Zou Y. DNA damage responses in progeroid syndromes arise from defective maturation of prelamin A.
    J Cell Sci. 2006 Nov 15;119(Pt 22):4644-4649. Epub 2006 Oct 24. PMID: 17062639 [PubMed - as supplied by publisher]
  76. Livneh H, Antonak RF, Maron S. Progeria: Medical.shtmlects, psychosocial perspectives, and intervention guidelines. Death Studies. 1995;19:433 - 452.
  77. Ly DH, Lockhart DJ, Lerner RA, Schultz PG. Mitotic misregulation and human aging. Science. 2000;287:2486-2492.
  78. Makous N, Friedman S, Yakovac W, al e. Cardiovascular manifestations in progeria. Report of clinical and pathologic findings in a patient with severe areteriosclerotic heart desease and aortic stenosis. Am. Heart J. 1962;64:334-346. (no abstract available online)
  79. Mallampalli MP, Huyer G, Bendale P, Gelb MH, Michaelis S. Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome. Proc Natl Acad Sci U S A. 2005 Oct 4;102(40):14416-21. Epub 2005 Sep 26. PMID: 16186497 [PubMed - indexed for MEDLINE]
  80. Mandera M, Larysz D, Pajak J, Klimczak A.                Related Articles, Links
    Epidural hematomas in a child with Hutchinson-Gilford progeria syndrome.
    Childs Nerv Syst. 2003 Jan;19(1):63-5.
    PMID: 12541091 [PubMed - indexed for MEDLINE]
  81. Martin GM. Genetic syndromes in man with potential relevance to the pathobiology of aging. Birth Defects Orig Artic Ser. 1978;14(1):5-39. Review. No abstract available. PMID: 147113 [PubMed - indexed for MEDLINE]
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  83. McClintock D, Gordon LB, Djabali K.
    Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody.
    Proc Natl Acad Sci U S A. 2006 Feb 14;103(7):2154-9. Epub 2006 Feb 6.
    PMID: 16461887 [PubMed - indexed for MEDLINE]
  84. Meta M, Yang SH, Bergo MO, Fong LG, Young SG. Protein farnesyltransferase inhibitors and progeria. Trends Mol Med. 2006 Oct;12(10):480-7. Epub 2006 Aug 30. PMID: 16942914 [PubMed - in process]
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  89. O'Brien ME, Weiss AS. Hutchinson-Gilford Progeria fibroblasts exhibit metabolically normal uridine uptake and RNA synthetic rates. Biochemical and Biophysical Research Communications. 1995;210(1):225 - 230.
  90. O'Brien ME, Weiss AS. Hutchinson-Gilford progeria: faithful DNA maintenance, inheritance and allelic transcription of B(1-4) galactosyltransferase. Mechanisms of Ageing and Development. 1998;101:43-56.
  91. O'Brien ME, Weiss AS. A novel B(1-4)galactosyltransferase gene silent mutation (594C>T) associated with Hutchinson-Gilford progeria. Hum Mutat. 2001 Apr;17(4):355. (no abstract available online)
  92. O'Neill M, Nunez F, Melton DW. 
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    p53 and a human premature ageing disorder.
    Mech Ageing Dev. 2003 May;124(5):599-603.
    PMID: 12735900 [PubMed - in process]
  93. Oshima J, Brown WT, Martin GM. No detectable mutations at Werner helicase locus in progeria. Lancet. 1996;348. (no abstract available online)
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  99. Reddel CJ, Weiss AS. Lamin A expression levels are unperturbed at the normal and mutant alleles but display partial splice site selection in Hutchinson-Gilford progeria syndrome.
    J Med Genet. 2004 Sep;41(9):715-7. PMID: 15342704 [PubMed - indexed for MEDLINE]
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  104. Robinson LJ, Karlsson NG, Weiss AS, Packer NH.
    Proteomic analysis of the genetic premature aging disease Hutchinson Gilford progeria syndrome reveals differential protein expression and glycosylation.
    J Proteome Res. 2003 Sep-Oct;2(5):556-7.
    PMID: 14582653 [PubMed - indexed for MEDLINE]
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  113. Rusinol AE, Sinensky MS. Farnesylated lamins, progeroid syndromes and farnesyl transferase inhibitors.
    J Cell Sci. 2006 Aug 15;119(Pt 16):3265-72. Review.
    PMID: 16899817 [PubMed - indexed for MEDLINE]
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    Nat Med. 2005 Apr;11(4):440-5. Epub 2005 Mar 6.
    PMID: 15750600 [PubMed - indexed for MEDLINE]
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