Mon-Li Chu

TitleProfessor
InstitutionThomas Jefferson University
DepartmentDermatology
Address233 S. 10th Street
Philadelphia PA 19107
Phone215-503-4834
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    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. Halabi CM, Broekelmann TJ, Lin M, Lee VS, Chu ML, Mecham RP. Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries. Sci Adv. 2017 May; 3(5):e1602532. PMID: 28508064.
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    2. Sasaki T, Hanisch FG, Deutzmann R, Sakai LY, Sakuma T, Miyamoto T, Yamamoto T, Hannappel E, Chu ML, Lanig H, von der Mark K. Functional consequence of fibulin-4 missense mutations associated with vascular and skeletal abnormalities and cutis laxa. Matrix Biol. 2016 Dec; 56:132-149. PMID: 27339457.
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    3. Khan SA, Dong H, Joyce J, Sasaki T, Chu ML, Tsuda T. Fibulin-2 is essential for angiotensin II-induced myocardial fibrosis mediated by transforming growth factor (TGF)-ß. Lab Invest. 2016 Jul; 96(7):773-83. PMID: 27111286.
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    4. Markova DZ, Pan TC, Zhang RZ, Zhang G, Sasaki T, Arita M, Birk DE, Chu ML. Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice. Cell Tissue Res. 2016 Jun; 364(3):637-46. PMID: 26711913.
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    5. Sasaki T, Stoop R, Sakai T, Hess A, Deutzmann R, Schlötzer-Schrehardt U, Chu ML, von der Mark K. Loss of fibulin-4 results in abnormal collagen fibril assembly in bone, caused by impaired lysyl oxidase processing and collagen cross-linking. Matrix Biol. 2016 Mar; 50:53-66. PMID: 26690653.
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    6. Igoucheva O, Alexeev V, Halabi CM, Adams SM, Stoilov I, Sasaki T, Arita M, Donahue A, Mecham RP, Birk DE, Chu ML. Fibulin-4 E57K Knock-in Mice Recapitulate Cutaneous, Vascular and Skeletal Defects of Recessive Cutis Laxa 1B with both Elastic Fiber and Collagen Fibril Abnormalities. J Biol Chem. 2015 Aug 28; 290(35):21443-59. PMID: 26178373.
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    7. Pan TC, Zhang RZ, Arita M, Bogdanovich S, Adams SM, Gara SK, Wagener R, Khurana TS, Birk DE, Chu ML. A Mouse Model for Dominant Collagen VI Disorders: HETEROZYGOUS DELETION OF Col6a3 EXON 16. J Biol Chem. 2014 Apr 11; 289(15):10293-307. PMID: 24563484.
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    8. Alexeev V, Arita M, Donahue A, Bonaldo P, Chu ML, Igoucheva O. Human adipose-derived stem cell transplantation as a potential therapy for collagen VI-related congenital muscular dystrophy. Stem Cell Res Ther. 2014; 5(1):21. PMID: 24522088.
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    9. Zhang H, Wu J, Dong H, Khan SA, Chu ML, Tsuda T. Fibulin-2 deficiency attenuates angiotensin II-induced cardiac hypertrophy by reducing transforming growth factor-ß signalling. Clin Sci (Lond). 2014 Feb; 126(4):275-88. PMID: 23841699.
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    10. Longmate WM, Monichan R, Chu ML, Tsuda T, Mahoney MG, DiPersio CM. Reduced Fibulin-2 Contributes to Loss of Basement Membrane Integrity and Skin Blistering in Mice Lacking Integrin a3ß1 in the Epidermis. J Invest Dermatol. 2014 Jun; 134(6):1609-17. PMID: 24390135.
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    11. Kang CY, Wang J, Axell-House D, Soni P, Chu ML, Chipitsyna G, Sarosiek K, Sendecki J, Hyslop T, Al-Zoubi M, Yeo CJ, Arafat HA. Clinical Significance of Serum COL6A3 in Pancreatic Ductal Adenocarcinoma. J Gastrointest Surg. 2014 Jan; 18(1):7-15. PMID: 24002763.
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    12. Pan TC, Zhang RZ, Markova D, Arita M, Zhang Y, Bogdanovich S, Khurana TS, Bönnemann CG, Birk DE, Chu ML. COL6A3 Protein Deficiency in Mice Leads to Muscle and Tendon Defects Similar to Human Collagen VI Congenital Muscular Dystrophy. J Biol Chem. 2013 May 17; 288(20):14320-31. PMID: 23564457.
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    13. Tsuda T, Wu J, Gao E, Joyce J, Markova D, Dong H, Liu Y, Zhang H, Zou Y, Gao F, Miller T, Koch W, Ma X, Chu ML. Loss of fibulin-2 protects against progressive ventricular dysfunction after myocardial infarction. J Mol Cell Cardiol. 2012 Jan; 52(1):273-82. PMID: 22100229.
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    14. Arafat H, Lazar M, Salem K, Chipitsyna G, Gong Q, Pan TC, Zhang RZ, Yeo CJ, Chu ML. Tumor-specific expression and alternative splicing of the COL6A3 gene in pancreatic cancer. Surgery. 2011 Aug; 150(2):306-15. PMID: 21719059.
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    15. Izu Y, Ansorge HL, Zhang G, Soslowsky LJ, Bonaldo P, Chu ML, Birk DE. Dysfunctional tendon collagen fibrillogenesis in collagen VI null mice. Matrix Biol. 2011 Jan; 30(1):53-61. PMID: 20951202.
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    16. Zhang RZ, Zou Y, Pan TC, Markova D, Fertala A, Hu Y, Squarzoni S, Reed UC, Marie SK, Bönnemann CG, Chu ML. Recessive COL6A2 C-globular missense mutations in Ullrich congenital muscular dystrophy: role of the C2a splice variant. J Biol Chem. 2010 Mar 26; 285(13):10005-15. PMID: 20106987.
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    17. Chapman SL, Sicot FX, Davis EC, Huang J, Sasaki T, Chu ML, Yanagisawa H. Fibulin-2 and fibulin-5 cooperatively function to form the internal elastic lamina and protect from vascular injury. Arterioscler Thromb Vasc Biol. 2010 Jan; 30(1):68-74. PMID: 19893004.
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    18. 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. PMID: 19349279.
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    19. Williams JA, Kondo N, Okabe T, Takeshita N, Pilchak DM, Koyama E, Ochiai T, Jensen D, Chu ML, Kane MA, Napoli JL, Enomoto-Iwamoto M, Ghyselinck N, Chambon P, Pacifici M, Iwamoto M. Retinoic acid receptors are required for skeletal growth, matrix homeostasis and growth plate function in postnatal mouse. Dev Biol. 2009 Apr 15; 328(2):315-27. PMID: 19389355.
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    20. Zou Y, Zhang RZ, Sabatelli P, Chu ML, Bönnemann CG. Muscle interstitial fibroblasts are the main source of collagen VI synthesis in skeletal muscle: implications for congenital muscular dystrophy types Ullrich and Bethlem. J Neuropathol Exp Neurol. 2008 Feb; 67(2):144-54. PMID: 18219255.
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    21. Sicot FX, Tsuda T, Markova D, Klement JF, Arita M, Zhang RZ, Pan TC, Mecham RP, Birk DE, Chu ML. Fibulin-2 is dispensable for mouse development and elastic fiber formation. Mol Cell Biol. 2008 Feb; 28(3):1061-7. PMID: 18070922.
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    22. Dasouki M, Markova D, Garola R, Sasaki T, Charbonneau NL, Sakai LY, Chu ML. Compound heterozygous mutations in fibulin-4 causing neonatal lethal pulmonary artery occlusion, aortic aneurysm, arachnodactyly, and mild cutis laxa. Am J Med Genet A. 2007 Nov 15; 143A(22):2635-41. PMID: 17937443.
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    23. Ji Y, Prasad NB, Novotny EA, Kaur S, Elkahloun A, Chen Y, Zhang RZ, Chu ML, Agarwal SK, Marx SJ, Collins FS, Chandrasekharappa SC. Mouse embryo fibroblasts lacking the tumor suppressor menin show altered expression of extracellular matrix protein genes. Mol Cancer Res. 2007 Oct; 5(10):1041-51. PMID: 17951404.
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    24. Petrini S, D'Amico A, Sale P, Lucarini L, Sabatelli P, Tessa A, Giusti B, Verardo M, Carrozzo R, Mattioli E, Scarpelli M, Chu ML, Pepe G, Russo MA, Bertini E. Ullrich myopathy phenotype with secondary ColVI defect identified by confocal imaging and electron microscopy analysis. Neuromuscul Disord. 2007 Aug; 17(8):587-96. PMID: 17588753.
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    25. Kobayashi N, Kostka G, Garbe JH, Keene DR, Bächinger HP, Hanisch FG, Markova D, Tsuda T, Timpl R, Chu ML, Sasaki T. A comparative analysis of the fibulin protein family. Biochemical characterization, binding interactions, and tissue localization. J Biol Chem. 2007 Apr 20; 282(16):11805-16. PMID: 17324935.
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    26. Chen SJ, Chu ML, Wang CJ, Liao CL, Hsieh SL, Sytwu HK, Wang CC. Kinetic Th1/Th2 responses of transgenic mice with bacterial meningitis induced by Haemophilus influenzae. Clin Sci (Lond). 2006 Oct; 111(4):253-63. PMID: 16623660.
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    27. Pepe G, Lucarini L, Zhang RZ, Pan TC, Giusti B, Quijano-Roy S, Gartioux C, Bushby KM, Guicheney P, Chu ML. COL6A1 genomic deletions in Bethlem myopathy and Ullrich muscular dystrophy. Ann Neurol. 2006 Jan; 59(1):190-5. PMID: 16278855.
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    28. Chen SJ, Liao CL, Hsieh SL, Chu ML, Fang MC, Sytwu HK, Wang CC. Kinetics of adaptive immunity to Haemophilus influenzae type b meningitis in transgenic mice: evidence from diverse expression of double T1/T2 transgenes and Th1/Th2-related cytokines. Immunol Lett. 2006 May 15; 105(1):6-15. PMID: 16384609.
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    29. Lucarini L, Giusti B, Zhang RZ, Pan TC, Jimenez-Mallebrera C, Mercuri E, Muntoni F, Pepe G, Chu ML. A homozygous COL6A2 intron mutation causes in-frame triple-helical deletion and nonsense-mediated mRNA decay in a patient with Ullrich congenital muscular dystrophy. Hum Genet. 2005 Sep; 117(5):460-6. PMID: 16075202.
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    30. Chu ML, Tsuda T. Fibulins in development and heritable disease. Birth Defects Res C Embryo Today. 2004 Mar; 72(1):25-36. PMID: 15054902.
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    31. Tsuda T, Markova D, Wang H, Evangelisti L, Pan TC, Chu ML. Zinc finger protein Zac1 is expressed in chondrogenic sites of the mouse. Dev Dyn. 2004 Feb; 229(2):340-8. PMID: 14745959.
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    32. Tsuda T, Gao E, Evangelisti L, Markova D, Ma X, Chu ML. Post-ischemic myocardial fibrosis occurs independent of hemodynamic changes. Cardiovasc Res. 2003 Oct 1; 59(4):926-33. PMID: 14553832.
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    33. Pan TC, Zhang RZ, Sudano DG, Marie SK, Bönnemann CG, Chu ML. New molecular mechanism for Ullrich congenital muscular dystrophy: a heterozygous in-frame deletion in the COL6A1 gene causes a severe phenotype. Am J Hum Genet. 2003 Aug; 73(2):355-69. PMID: 12840783.
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    34. Timpl R, Sasaki T, Kostka G, Chu ML. Fibulins: a versatile family of extracellular matrix proteins. Nat Rev Mol Cell Biol. 2003 Jun; 4(6):479-89. PMID: 12778127.
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    35. Markova D, Zou Y, Ringpfeil F, Sasaki T, Kostka G, Timpl R, Uitto J, Chu ML. Genetic heterogeneity of cutis laxa: a heterozygous tandem duplication within the fibulin-5 (FBLN5) gene. Am J Hum Genet. 2003 Apr; 72(4):998-1004. PMID: 12618961.
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    36. Tsuda T, Pan TC, Evangelisti L, Chu ML. Prominent expression of lysyl oxidase during mouse embryonic cardiovascular development. Anat Rec A Discov Mol Cell Evol Biol. 2003 Feb; 270(2):93-6. PMID: 12524683.
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    37. Zhang RZ, Sabatelli P, Pan TC, Squarzoni S, Mattioli E, Bertini E, Pepe G, Chu ML. Effects on collagen VI mRNA stability and microfibrillar assembly of three COL6A2 mutations in two families with Ullrich congenital muscular dystrophy. J Biol Chem. 2002 Nov 15; 277(46):43557-64. PMID: 12218063.
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    38. Vanegas OC, Zhang RZ, Sabatelli P, Lattanzi G, Bencivenga P, Giusti B, Columbaro M, Chu ML, Merlini L, Pepe G. Novel COL6A1 splicing mutation in a family affected by mild Bethlem myopathy. Muscle Nerve. 2002 Apr; 25(4):513-9. PMID: 11932968.
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    39. Dziadek M, Kazenwadel JS, Hendrey JA, Pan TC, Zhang RZ, Chu ML. Alternative splicing of transcripts for the alpha 3 chain of mouse collagen VI: identification of an abundant isoform lacking domains N7-N10 in mouse and human. Matrix Biol. 2002 Apr; 21(3):227-41. PMID: 12009329.
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    40. Pepe G, de Visser M, Bertini E, Bushby K, Vanegas OC, Chu ML, Lattanzi G, Merlini L, Muntoni F, Urtizberea A. Bethlem myopathy (BETHLEM) 86th ENMC international workshop, 10-11 November 2000, Naarden, The Netherlands. Neuromuscul Disord. 2002 Mar; 12(3):296-305. PMID: 11801404.
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    41. Castoldi M, Chu ML. Structural and functional characterization of the human and mouse fibulin-1 gene promoters: role of Sp1 and Sp3. Biochem J. 2002 Feb 15; 362(Pt 1):41-50. PMID: 11829738.
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