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Bone marrow transplant with post-transplant cyclophosphamide for recessive dystrophic epidermolysis bullosa expands the related donor pool and permits tolerance of non-hematopoietic cellular grafts.

Ebens CL, McGrath JA, Tamai K, Hovnanian A, Wagner JE, Riddle MJ, Keene DR, DeFor TE, Tryon R, Chen M, Woodley DT, Hook K, Tolar J.

Br J Dermatol. 2019 Mar 6. doi: 10.1111/bjd.17858. [Epub ahead of print]


Fibrillin protein pleiotropy: Acromelic dysplasias.

Sakai LY, Keene DR.

Matrix Biol. 2018 Sep 13. pii: S0945-053X(18)30312-3. doi: 10.1016/j.matbio.2018.09.005. [Epub ahead of print] Review.


Correction: Sex, pregnancy and aortic disease in Marfan syndrome.

Renard M, Muiño-Mosquera L, Manalo EC, Tufa S, Carlson EJ, Keene DR, Backer J, Sakai LY.

PLoS One. 2018 May 14;13(5):e0197631. doi: 10.1371/journal.pone.0197631. eCollection 2018.


Surface-exposed loops and an acidic patch in the Scl1 protein of group A Streptococcus enable Scl1 binding to wound-associated fibronectin.

McNitt DH, Choi SJ, Keene DR, Van De Water L, Squeglia F, Berisio R, Lukomski S.

J Biol Chem. 2018 May 18;293(20):7796-7810. doi: 10.1074/jbc.RA118.002250. Epub 2018 Apr 2.


Type III collagen affects dermal and vascular collagen fibrillogenesis and tissue integrity in a mutant Col3a1 transgenic mouse model.

D'hondt S, Guillemyn B, Syx D, Symoens S, De Rycke R, Vanhoutte L, Toussaint W, Lambrecht BN, De Paepe A, Keene DR, Ishikawa Y, Bächinger HP, Janssens S, Bertrand MJM, Malfait F.

Matrix Biol. 2018 Sep;70:72-83. doi: 10.1016/j.matbio.2018.03.008. Epub 2018 Mar 15.


Ultrastructural analysis of the extracellular matrix.

Keene DR, Tufa SF.

Methods Cell Biol. 2018;143:1-39. doi: 10.1016/bs.mcb.2017.08.002. Epub 2017 Oct 25.


mTORC1 Signaling is a Critical Regulator of Postnatal Tendon Development.

Lim J, Munivez E, Jiang MM, Song IW, Gannon F, Keene DR, Schweitzer R, Lee BH, Joeng KS.

Sci Rep. 2017 Dec 7;7(1):17175. doi: 10.1038/s41598-017-17384-0.


Optimizing a 3D model system for molecular manipulation of tenogenesis.

Chien C, Pryce B, Tufa SF, Keene DR, Huang AH.

Connect Tissue Res. 2018 Jul;59(4):295-308. doi: 10.1080/03008207.2017.1383403. Epub 2017 Oct 13.


Fibulin-4 deposition requires EMILIN-1 in the extracellular matrix of osteoblasts.

Schiavinato A, Keene DR, Imhof T, Doliana R, Sasaki T, Sengle G.

Sci Rep. 2017 Jul 17;7(1):5526. doi: 10.1038/s41598-017-05835-7.


Sex, pregnancy and aortic disease in Marfan syndrome.

Renard M, Muiño-Mosquera L, Manalo EC, Tufa S, Carlson EJ, Keene DR, De Backer J, Sakai LY.

PLoS One. 2017 Jul 14;12(7):e0181166. doi: 10.1371/journal.pone.0181166. eCollection 2017. Erratum in: PLoS One. 2018 May 14;13(5):e0197631.


Cell Adhesion Molecule CD166/ALCAM Functions Within the Crypt to Orchestrate Murine Intestinal Stem Cell Homeostasis.

Smith NR, Davies PS, Levin TG, Gallagher AC, Keene DR, Sengupta SK, Wieghard N, El Rassi E, Wong MH.

Cell Mol Gastroenterol Hepatol. 2017 Jan 25;3(3):389-409. doi: 10.1016/j.jcmgh.2016.12.010. eCollection 2017 May.


Novel Model of Tendon Regeneration Reveals Distinct Cell Mechanisms Underlying Regenerative and Fibrotic Tendon Healing.

Howell K, Chien C, Bell R, Laudier D, Tufa SF, Keene DR, Andarawis-Puri N, Huang AH.

Sci Rep. 2017 Mar 23;7:45238. doi: 10.1038/srep45238.


Fkbp10 Deletion in Osteoblasts Leads to Qualitative Defects in Bone.

Lietman CD, Lim J, Grafe I, Chen Y, Ding H, Bi X, Ambrose CG, Fratzl-Zelman N, Roschger P, Klaushofer K, Wagermaier W, Schmidt I, Fratzl P, Rai J, Weis M, Eyre D, Keene DR, Krakow D, Lee BH.

J Bone Miner Res. 2017 Jun;32(6):1354-1367. doi: 10.1002/jbmr.3108. Epub 2017 Mar 20.


EMILIN3, an extracellular matrix molecule with restricted distribution in skin.

Corallo D, Schiavinato A, Bizzotto D, Milanetto M, Guljelmovic M, Keene DR, Sengle G, Braghetta P, Bonaldo P.

Exp Dermatol. 2017 May;26(5):435-438. doi: 10.1111/exd.13254. Epub 2017 Mar 2.


Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis Bullosa.

Siprashvili Z, Nguyen NT, Gorell ES, Loutit K, Khuu P, Furukawa LK, Lorenz HP, Leung TH, Keene DR, Rieger KE, Khavari P, Lane AT, Tang JY, Marinkovich MP.

JAMA. 2016 Nov 1;316(17):1808-1817. doi: 10.1001/jama.2016.15588.


Unique Footprint in the scl1.3 Locus Affects Adhesion and Biofilm Formation of the Invasive M3-Type Group A Streptococcus.

Bachert BA, Choi SJ, LaSala PR, Harper TI, McNitt DH, Boehm DT, Caswell CC, Ciborowski P, Keene DR, Flores AR, Musser JM, Squeglia F, Marasco D, Berisio R, Lukomski S.

Front Cell Infect Microbiol. 2016 Aug 31;6:90. doi: 10.3389/fcimb.2016.00090. eCollection 2016.


FBN1: The disease-causing gene for Marfan syndrome and other genetic disorders.

Sakai LY, Keene DR, Renard M, De Backer J.

Gene. 2016 Oct 10;591(1):279-291. doi: 10.1016/j.gene.2016.07.033. Epub 2016 Jul 18. Review.


BMP1-like proteinases are essential to the structure and wound healing of skin.

Muir AM, Massoudi D, Nguyen N, Keene DR, Lee SJ, Birk DE, Davidson JM, Marinkovich MP, Greenspan DS.

Matrix Biol. 2016 Dec;56:114-131. doi: 10.1016/j.matbio.2016.06.004. Epub 2016 Jun 27.


Targeting of EMILIN-1 and EMILIN-2 to Fibrillin Microfibrils Facilitates their Incorporation into the Extracellular Matrix.

Schiavinato A, Keene DR, Wohl AP, Corallo D, Colombatti A, Wagener R, Paulsson M, Bonaldo P, Sengle G.

J Invest Dermatol. 2016 Jun;136(6):1150-1160. doi: 10.1016/j.jid.2016.02.021. Epub 2016 Mar 2.


Molecular Mechanism Responsible for Fibronectin-controlled Alterations in Matrix Stiffness in Advanced Chronic Liver Fibrogenesis.

Iwasaki A, Sakai K, Moriya K, Sasaki T, Keene DR, Akhtar R, Miyazono T, Yasumura S, Watanabe M, Morishita S, Sakai T.

J Biol Chem. 2016 Jan 1;291(1):72-88. doi: 10.1074/jbc.M115.691519. Epub 2015 Nov 9.


Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.

Sengle G, Carlberg V, Tufa SF, Charbonneau NL, Smaldone S, Carlson EJ, Ramirez F, Keene DR, Sakai LY.

PLoS Genet. 2015 Jun 26;11(6):e1005340. doi: 10.1371/journal.pgen.1005340. eCollection 2015 Jun.


Musculoskeletal integration at the wrist underlies the modular development of limb tendons.

Huang AH, Riordan TJ, Pryce B, Weibel JL, Watson SS, Long F, Lefebvre V, Harfe BD, Stadler HS, Akiyama H, Tufa SF, Keene DR, Schweitzer R.

Development. 2015 Jul 15;142(14):2431-41. doi: 10.1242/dev.122374. Epub 2015 Jun 10.


Intermolecular interactions of thrombospondins drive their accumulation in extracellular matrix.

Kim DJ, Christofidou ED, Keene DR, Hassan Milde M, Adams JC.

Mol Biol Cell. 2015 Jul 15;26(14):2640-54. doi: 10.1091/mbc.E14-05-0996. Epub 2015 May 20.


A review of color blindness for microscopists: guidelines and tools for accommodating and coping with color vision deficiency.

Keene DR.

Microsc Microanal. 2015 Apr;21(2):279-89. doi: 10.1017/S1431927615000173. Epub 2015 Mar 5. Review.


Correlation of the same fields imaged in the TEM, confocal, LM, and microCT by image registration: from specimen preparation to displaying a final composite image.

Keene DR, Tufa SF, Wong MH, Smith NR, Sakai LY, Horton WA.

Methods Cell Biol. 2014;124:391-417. doi: 10.1016/B978-0-12-801075-4.00018-5.


Somatic correction of junctional epidermolysis bullosa by a highly recombinogenic AAV variant.

Melo SP, Lisowski L, Bashkirova E, Zhen HH, Chu K, Keene DR, Marinkovich MP, Kay MA, Oro AE.

Mol Ther. 2014 Apr;22(4):725-33. doi: 10.1038/mt.2013.290. Epub 2014 Jan 6.


A trans-acting protein effect causes severe eye malformation in the Mp mouse.

Rainger J, Keighren M, Keene DR, Charbonneau NL, Rainger JK, Fisher M, Mella S, Huang JT, Rose L, van't Hof R, Sakai LY, Jackson IJ, Fitzpatrick DR.

PLoS Genet. 2013;9(12):e1003998. doi: 10.1371/journal.pgen.1003998. Epub 2013 Dec 12.


Patient-specific naturally gene-reverted induced pluripotent stem cells in recessive dystrophic epidermolysis bullosa.

Tolar J, McGrath JA, Xia L, Riddle MJ, Lees CJ, Eide C, Keene DR, Liu L, Osborn MJ, Lund TC, Blazar BR, Wagner JE.

J Invest Dermatol. 2014 May;134(5):1246-1254. doi: 10.1038/jid.2013.523. Epub 2013 Dec 6.


Nonselective assembly of fibrillin 1 and fibrillin 2 in the rodent ocular zonule and in cultured cells: implications for Marfan syndrome.

Beene LC, Wang LW, Hubmacher D, Keene DR, Reinhardt DP, Annis DS, Mosher DF, Mecham RP, Traboulsi EI, Apte SS.

Invest Ophthalmol Vis Sci. 2013 Dec 23;54(13):8337-44. doi: 10.1167/iovs.13-13121.


AMACO is a component of the basement membrane-associated Fraser complex.

Richardson RJ, Gebauer JM, Zhang JL, Kobbe B, Keene DR, Karlsen KR, Richetti S, Wohl AP, Sengle G, Neiss WF, Paulsson M, Hammerschmidt M, Wagener R.

J Invest Dermatol. 2014 May;134(5):1313-1322. doi: 10.1038/jid.2013.492. Epub 2013 Nov 14.


De novo anti-type VII collagen antibodies in patients with recessive dystrophic epidermolysis bullosa.

Woodley DT, Cogan J, Wang X, Hou Y, Haghighian C, Kudo G, Keene DR, Chen M.

J Invest Dermatol. 2014 Apr;134(4):1138-1140. doi: 10.1038/jid.2013.475. Epub 2013 Nov 8. No abstract available.


Type 2 alveolar cells are stem cells in adult lung.

Barkauskas CE, Cronce MJ, Rackley CR, Bowie EJ, Keene DR, Stripp BR, Randell SH, Noble PW, Hogan BL.

J Clin Invest. 2013 Jul;123(7):3025-36. doi: 10.1172/JCI68782. Epub 2013 Jun 10.


Posttranslational modifications in type I collagen from different tissues extracted from wild type and prolyl 3-hydroxylase 1 null mice.

Pokidysheva E, Zientek KD, Ishikawa Y, Mizuno K, Vranka JA, Montgomery NT, Keene DR, Kawaguchi T, Okuyama K, Bächinger HP.

J Biol Chem. 2013 Aug 23;288(34):24742-52. doi: 10.1074/jbc.M113.464156. Epub 2013 Jul 16.


A correlative method for imaging identical regions of samples by micro-CT, light microscopy, and electron microscopy: imaging adipose tissue in a model system.

Sengle G, Tufa SF, Sakai LY, Zulliger MA, Keene DR.

J Histochem Cytochem. 2013 Apr;61(4):263-71. doi: 10.1369/0022155412473757. Epub 2012 Dec 20.


Keratinocyte-targeted expression of human laminin γ2 rescues skin blistering and early lethality of laminin γ2 deficient mice.

Adair-Kirk TL, Griffin GL, Meyer MJ, Kelley DG, Miner JH, Keene DR, Marinkovich MP, Ruppert JM, Uitto J, Senior RM.

PLoS One. 2012;7(9):e45546. doi: 10.1371/journal.pone.0045546. Epub 2012 Sep 18.


Keratinocytes from induced pluripotent stem cells in junctional epidermolysis bullosa.

Tolar J, Xia L, Lees CJ, Riddle M, McElroy A, Keene DR, Lund TC, Osborn MJ, Marinkovich MP, Blazar BR, Wagner JE.

J Invest Dermatol. 2013 Feb;133(2):562-5. doi: 10.1038/jid.2012.278. Epub 2012 Aug 30. No abstract available.


Disruption of a Sox9-β-catenin circuit by mutant Fgfr3 in thanatophoric dysplasia type II.

Shung CY, Ota S, Zhou ZQ, Keene DR, Hurlin PJ.

Hum Mol Genet. 2012 Nov 1;21(21):4628-44. doi: 10.1093/hmg/dds305. Epub 2012 Jul 26.


Collagen XII and XIV, new partners of cartilage oligomeric matrix protein in the skin extracellular matrix suprastructure.

Agarwal P, Zwolanek D, Keene DR, Schulz JN, Blumbach K, Heinegård D, Zaucke F, Paulsson M, Krieg T, Koch M, Eckes B.

J Biol Chem. 2012 Jun 29;287(27):22549-59. doi: 10.1074/jbc.M111.335935. Epub 2012 May 9.


Mutation in cyclophilin B that causes hyperelastosis cutis in American Quarter Horse does not affect peptidylprolyl cis-trans isomerase activity but shows altered cyclophilin B-protein interactions and affects collagen folding.

Ishikawa Y, Vranka JA, Boudko SP, Pokidysheva E, Mizuno K, Zientek K, Keene DR, Rashmir-Raven AM, Nagata K, Winand NJ, Bächinger HP.

J Biol Chem. 2012 Jun 22;287(26):22253-65. doi: 10.1074/jbc.M111.333336. Epub 2012 May 3.


Microenvironmental regulation by fibrillin-1.

Sengle G, Tsutsui K, Keene DR, Tufa SF, Carlson EJ, Charbonneau NL, Ono RN, Sasaki T, Wirtz MK, Samples JR, Fessler LI, Fessler JH, Sekiguchi K, Hayflick SJ, Sakai LY.

PLoS Genet. 2012 Jan;8(1):e1002425. doi: 10.1371/journal.pgen.1002425. Epub 2012 Jan 5.


Conversion of mechanical force into TGF-β-mediated biochemical signals.

Maeda T, Sakabe T, Sunaga A, Sakai K, Rivera AL, Keene DR, Sasaki T, Stavnezer E, Iannotti J, Schweitzer R, Ilic D, Baskaran H, Sakai T.

Curr Biol. 2011 Jun 7;21(11):933-41. doi: 10.1016/j.cub.2011.04.007. Epub 2011 May 19.


Sequential and coordinated actions of c-Myc and N-Myc control appendicular skeletal development.

Zhou ZQ, Shung CY, Ota S, Akiyama H, Keene DR, Hurlin PJ.

PLoS One. 2011 Apr 11;6(4):e18795. doi: 10.1371/journal.pone.0018795.


ADAMTS10 protein interacts with fibrillin-1 and promotes its deposition in extracellular matrix of cultured fibroblasts.

Kutz WE, Wang LW, Bader HL, Majors AK, Iwata K, Traboulsi EI, Sakai LY, Keene DR, Apte SS.

J Biol Chem. 2011 May 13;286(19):17156-67. doi: 10.1074/jbc.M111.231571. Epub 2011 Mar 14.


A fibronectin-independent mechanism of collagen fibrillogenesis in adult liver remodeling.

Moriya K, Bae E, Honda K, Sakai K, Sakaguchi T, Tsujimoto I, Kamisoyama H, Keene DR, Sasaki T, Sakai T.

Gastroenterology. 2011 May;140(5):1653-63. doi: 10.1053/j.gastro.2011.02.005. Epub 2011 Feb 12.


Transmission electron microscopy of cartilage and bone.

Keene DR, Tufa SF.

Methods Cell Biol. 2010;96:443-73. doi: 10.1016/S0091-679X(10)96019-6. Review.


Molecular imaging of activated von Willebrand factor to detect high-risk atherosclerotic phenotype.

McCarty OJ, Conley RB, Shentu W, Tormoen GW, Zha D, Xie A, Qi Y, Zhao Y, Carr C, Belcik T, Keene DR, de Groot PG, Lindner JR.

JACC Cardiovasc Imaging. 2010 Sep;3(9):947-55. doi: 10.1016/j.jcmg.2010.06.013.


Bone marrow transplantation for recessive dystrophic epidermolysis bullosa.

Wagner JE, Ishida-Yamamoto A, McGrath JA, Hordinsky M, Keene DR, Woodley DT, Chen M, Riddle MJ, Osborn MJ, Lund T, Dolan M, Blazar BR, Tolar J.

N Engl J Med. 2010 Aug 12;363(7):629-39. doi: 10.1056/NEJMoa0910501. Erratum in: N Engl J Med. 2010 Sep 30;363(14):1383. Woodley, David T [added]; Chen, Mei [added].


The atypical homeodomain transcription factor Mohawk controls tendon morphogenesis.

Liu W, Watson SS, Lan Y, Keene DR, Ovitt CE, Liu H, Schweitzer R, Jiang R.

Mol Cell Biol. 2010 Oct;30(20):4797-807. doi: 10.1128/MCB.00207-10. Epub 2010 Aug 9.


Nano-structure of the laminin γ-1 short arm reveals an extended and curved multidomain assembly.

Patel TR, Morris GA, Zwolanek D, Keene DR, Li J, Harding SE, Koch M, Stetefeld J.

Matrix Biol. 2010 Sep;29(7):565-72. doi: 10.1016/j.matbio.2010.07.004. Epub 2010 Aug 3.


In vivo studies of mutant fibrillin-1 microfibrils.

Charbonneau NL, Carlson EJ, Tufa S, Sengle G, Manalo EC, Carlberg VM, Ramirez F, Keene DR, Sakai LY.

J Biol Chem. 2010 Aug 6;285(32):24943-55. doi: 10.1074/jbc.M110.130021. Epub 2010 Jun 7.

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