Selected Publications

Cook JR, Smaldone S, Cozzolino C, Del Solar M, Lee-Arteaga S, Nistala H, Ramirez F. Generation ofFbn1 conditional null mice implicates the extracellular microfibrils in osteoprogenitor recruitment. Genesis. 2012 Feb 28.

Smaldone S, Carta L, Ramirez F. Establishment of fibrillin-deficient osteoprogenitor cell lines identifies molecular abnormalities associated with extracellular matrix perturbation of osteogenic differentiation. Cell Tissue Res. 2011 Jun;344(3):511-7.

Arteaga-Solis E, Sui-Arteaga L, Kim M, Schaffler MB, Jepsen KJ, Pleshko N, Ramirez F. Material and mechanical properties of bones deficient for fibrillin-1 or fibrillin-2 microfibrils. Matrix Biol. 2011 Apr;30(3):188-94.

Smaldone S, Olivieri J, Gusella GL, Moroncini G, Gabrielli A, Ramirez F. Ha-Ras stabilization mediates pro-fibrotic signals in dermal fibroblasts. Fibrogenesis Tissue Repair. 2011 Mar 1;4(1):8. 

Olivieri J, Smaldone S, Ramirez F. Fibrillin assemblies: extracellular determinants of tissue formation and fibrosis. Fibrogenesis Tissue Repair. 2010 Dec 2;3:24.

Nistala H, Lee-Arteaga S, Carta L, Cook JR, Smaldone S, Siciliano G, Rifkin AN, Dietz HC, Rifkin DB, Ramirez F. Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndrome. Hum Mol Genet. 2010 Dec 15;19(24):4790-8.

Nistala H, Lee-Arteaga S, Smaldone S, Siciliano G, Carta L, Ono RN, Sengle G, Arteaga-Solis E, Levasseur R, Ducy P, Sakai LY, Karsenty G, Ramirez F. Fibrillin-1 and -2 differentially modulate endogenous TGF-{beta} and BMP bioavailability during bone formation. J Cell Biol. 2010 Sep 20;190(6):1107-21.

Nistala H, Lee-Arteaga S, Smaldone S, Siciliano G, Ramirez F. Extracellular microfibrils modulate osteoblast-supported osteoclastogenesis by restricting TGF beta stimulation of RANKL production. J Biol Chem.2010 Oct 29;285(44):34126-33.

Cook JR, Nistala H, Ramirez F. Drug-based therapies for vascular disease in Marfan syndrome: from mouse models to human patients. Mt Sinai J Med. 2010 Jul-Aug;77(4):366-73.

Charbonneau NL, Carlson EJ, Tufa S, Sengle G, Manalo EC, Carlberg VM, Ramirez F, Keene DR, Sakai LY.  In vivo studies of mutant fibrillin-1 microfibrils. J Biol Chem. 2010 Aug 6;285(32):24943-55.

Charbonneau NL, Jordan CD, Keene DR, Lee-Arteaga S, Dietz HC, Rifkin DB, Ramirez F, Sakai LY. Microfibril structure masks fibrillin-2 in postnatal tissues. J Biol Chem. 2010 Jun 25;285(26):20242-51.

Chernousov MA, Baylor K, Stahl RC, Stecker MM, Sakai LY, Lee-Arteaga S, Ramirez F, Carey DJ.  Fibrillin-2 is dispensable for peripheral nerve development, myelination and regeneration. Matrix Biol. 2010 Jun;29(5):357-68.

Nistala H, Lee-Arteaga S, Siciliano G, Smaldone S, Ramirez F. Extracellular regulation of transforming growth factor beta and bone morphogenetic protein signaling in bone. Ann N Y Acad Sci. 2010 Mar;1192(1):253-6.

Su J, Gorse K, Ramirez F, Fox MA. Collagen XIX is expressed by interneurons and contributes to the formation of hippocampal synapses. J Comp Neurol. 2010 Jan 10;518(2):229-53.

Ramirez F, Sakai LY. Biogenesis and function of fibrillin assemblies. Cell Tissue Res. 2010 Jan;339(1):71-82.  

Carta L, Wagenseil JE, Knutsen RH, Mariko B, Faury G, Davis EC, Starcher B, Mecham RP, Ramirez F. Discrete contributions of elastic fiber components to arterial development and mechanical compliance. Arterioscler Thromb Vasc Biol. 2009 Dec;29(12):2083-9.

Ramirez F, Rifkin DB. Extracellular microfibrils: contextual platforms for TGFbeta and BMP signaling. Curr Opin Cell Biol. 2009 Oct;21(5):616-22.

Ono RN, Sengle G, Charbonneau NL, Carlberg V, Bächinger HP, Sasaki T, Lee-Arteaga S, Zilberberg L, Rifkin DB, Ramirez F, Chu ML, Sakai LY. Latent transforming growth factor beta-binding proteins and fibulins compete for fibrillin-1 and exhibit exquisite specificities in binding sites. J Biol Chem. 2009 Jun 19;284(25):16872-81.

Ramirez F, Dietz HC. Extracellular microfibrils in vertebrate development and disease processes. J Biol Chem. 2009 May 29;284(22):14677-81.

Carta L, Smaldone S, Zilberberg L, Loch D, Dietz HC, Rifkin DB, Ramirez F. p38 MAPK is an early determinant of promiscuous Smad2/3 signaling in the aortas of fibrillin-1 (Fbn1)-null mice. J Biol Chem. 2009 Feb 27;284(9):5630-6.

Dabovic B, Chen Y, Choi J, Vassallo M, Dietz HC, Ramirez F, von Melchner H, Davis EC, Rifkin DB. Dual functions for LTBP in lung development: LTBP-4 independently modulates elastogenesis and TGF-beta activity. J Cell Physiol. 2009 Apr;219(1):14-22.

Matsuo N, Tanaka S, Yoshioka H, Koch M, Gordon MK, Ramirez F. Collagen XXIV (Col24a1) gene expression is a specific marker of osteoblast differentiation and bone formation. Connect Tissue Res. 2008;49(2):68-75.

Ramirez F, Carta L, Lee-Arteaga S, Liu C, Nistala H, Smaldone S. Fibrillin-rich microfibrils - structural and instructive determinants of mammalian development and physiology. Connect Tissue Res. 2008;49(1):1-6.

Ramirez F, Dietz HC. Marfan syndrome: from molecular pathogenesis to clinical treatment. Curr Opin Genet Dev. 2007 Jun;17(3):252-8.

Cohn RD, van Erp C, Habashi JP, Soleimani AA, Klein EC, Lisi MT, Gamradt M, ap Rhys CM, Holm TM, Loeys BL, Ramirez F, Judge DP, Ward CW, Dietz HC. Angiotensin II type 1 receptor blockade attenuates TGF-beta-induced failure of muscle regeneration in multiple myopathic states. Nat Med. 2007 Feb;13(2):204-10.

Kavukcuoglu B, Arteaga-Solis E, Lee-Arteaga S, Ramirez F and Mann AB.  Nanomechanics and Raman Spectroscopy of fibrillin 2 knock-out mouse bones.  J. Mater. Sci. 2007; 42:8788-8794.

Habashi JP, Judge DP, Holm TM, Cohn RD, Loeys BL, Cooper TK, Myers L, Klein EC, Liu G, Calvi C, Podowski M, Neptune ER, Halushka MK, Bedja D, Gabrielson K, Rifkin DB, Carta L, Ramirez F, Huso DL, Dietz HC. Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science. 2006 Apr 7;312(5770):117-21.

Carta L, Pereira L, Arteaga-Solis E, Lee-Arteaga SY, Lenart B, Starcher B, Merkel CA, Sukoyan M, Kerkis A, Hazeki N, Keene DR, Sakai LY, Ramirez F. Fibrillins 1 and 2 perform partially overlapping functions during aortic development. J Biol Chem. 2006 Mar 24;281(12):8016-23.

Lei L, Laub F, Lush M, Romero M, Zhou J, Luikart B, Klesse L, Ramirez F, Parada LF. The zinc finger transcription factor Klf7 is required for TrkA gene expression and development of nociceptive sensory neurons. Genes Dev. 2005 Jun 1;19(11):1354-64.

Laub F, Lei L, Sumiyoshi H, Kajimura D, Dragomir C, Smaldone S, Puche AC, Petros TJ, Mason C, Parada LF, Ramirez F. Transcription factor KLF7 is important for neuronal morphogenesis in selected regions of the nervous system. Mol Cell Biol. 2005 Jul;25(13):5699-711.

Tanaka S, Antoniv TT, Liu K, Wang L, Wells DJ, Ramirez F, Bou-Gharios G. Cooperativity between far upstream enhancer and proximal promoter elements of the human {alpha}2(I) collagen (COL1A2) gene instructs tissue specificity in transgenic mice. J Biol Chem. 2004 Dec 31;279(53):56024-31.

Sumiyoshi H, Mor N, Lee SY, Doty S, Henderson S, Tanaka S, Yoshioka H, Rattan S, Ramirez F. Esophageal muscle physiology and morphogenesis require assembly of a collagen XIX-rich basement membrane zone. J Cell Biol. 2004 Aug 16;166(4):591-600.

Chanut-Delalande H, Bonod-Bidaud C, Cogne S, Malbouyres M, Ramirez F, Fichard A, Ruggiero F. Development of a functional skin matrix requires deposition of collagen V heterotrimers. Mol Cell Biol. 2004 Jul;24(13):6049-57.

Smaldone S, Laub F, Else C, Dragomir C, Ramirez F. Identification of MoKA, a novel F-box protein that modulates Krüppel-like transcription factor 7 activity. Mol Cell Biol. 2004 Feb;24(3):1058-69.

Ramirez F, Dietz HC. Therapy insight: aortic aneurysm and dissection in Marfan's syndrome. Nat Clin Pract Cardiovasc Med. 2004 Nov;1(1):31-6.

Chen Y, Sumiyoshi H, Oxford JT, Yoshioka H, Ramirez F, Morris NP. Cis-acting elements regulate alternative splicing of exons 6A, 6B and 8 of the alpha1(XI) collagen gene and contribute to the regional diversification of collagen XI matrices. Matrix Biol. 2001 Dec;20(8):589-99.

Arteaga-Solis E, Gayraud B, Lee SY, Shum L, Sakai L, Ramirez F. Regulation of limb patterning by extracellular microfibrils. J Cell Biol. 2001 Jul 23;154(2):275-81.

Laub F, Aldabe R, Ou J, Ramirez F. Overexpression of a novel zinc-finger protein induces apoptosis in NIH3T3 fibroblasts. Genomics. 2000 Dec 15;70(3):375-80.


Contact Us

Francesco Ramirez, DSc
Principal Investigator
Tel: 212-241-7237
Fax: 212-996-7214
Send e-mail

Karen J. Johnson
Administrative Coordinator
Tel: 212-241-7245
Fax: 212-996-7214
Send e-mail

Pharmacology and Systems Therapeutics
1468 Madison Avenue
New York, NY 10029