메뉴 건너뛰기




Volumn 17, Issue 2, 2009, Pages 253-261

Cardiac expression of a mini-dystrophin that normalizes skeletal muscle force only partially restores heart function in aged Mdx mice

Author keywords

[No Author keywords available]

Indexed keywords

CARDIOVASCULAR AGENT; DELTAH2 R19 MINI DYSTROPHIN; DOBUTAMINE; MYOSIN HEAVY CHAIN ALPHA; UNCLASSIFIED DRUG; DYSTROPHIN;

EID: 63649114966     PISSN: 15250016     EISSN: 15250024     Source Type: Journal    
DOI: 10.1038/mt.2008.264     Document Type: Article
Times cited : (78)

References (45)
  • 1
    • 33644670773 scopus 로고    scopus 로고
    • Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy
    • American Academy of Pediatrics Section on Cardiology and Cardiac Surgery
    • American Academy of Pediatrics Section on Cardiology and Cardiac Surgery (2005). Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy. Pediatrics 116: 1569-1573.
    • (2005) Pediatrics , vol.116 , pp. 1569-1573
  • 2
    • 33645739391 scopus 로고    scopus 로고
    • Treatment of the heart in Duchenne muscular dystrophy
    • Baxter, P (2006). Treatment of the heart in Duchenne muscular dystrophy. Dev Med Child Neurol 48: 163.
    • (2006) Dev Med Child Neurol , vol.48 , pp. 163
    • Baxter, P.1
  • 3
    • 33749004495 scopus 로고    scopus 로고
    • Challenges and opportunities in dystrophin-deficient cardiomyopathy gene therapy
    • spec. 2
    • Duan, D (2006). Challenges and opportunities in dystrophin-deficient cardiomyopathy gene therapy. Hum Mol Genet 15 (spec. 2): R253-R261.
    • (2006) Hum Mol Genet , vol.15
    • Duan, D.1
  • 4
    • 33846271135 scopus 로고    scopus 로고
    • Dystrophin, its interactions with other proteins, and implications for muscular dystrophy
    • Ervasti, JM (2007). Dystrophin, its interactions with other proteins, and implications for muscular dystrophy. Biochim Biophys Acta 1772: 108-117.
    • (2007) Biochim Biophys Acta , vol.1772 , pp. 108-117
    • Ervasti, J.M.1
  • 5
    • 0036566558 scopus 로고    scopus 로고
    • Expression of Dp260 in muscle tethers the actin cytoskeleton to the dystrophin-glycoprotein complex and partially prevents dystrophy
    • Warner, LE, DelloRusso, C, Crawford, RW, Rybakova, IN, Patel, JR, Ervasti, JM et al. (2002). Expression of Dp260 in muscle tethers the actin cytoskeleton to the dystrophin-glycoprotein complex and partially prevents dystrophy. Hum Mol Genet 11: 1095-1105.
    • (2002) Hum Mol Genet , vol.11 , pp. 1095-1105
    • Warner, L.E.1    DelloRusso, C.2    Crawford, R.W.3    Rybakova, I.4    Patel, J.R.5    Ervasti, J.M.6
  • 6
    • 0034683567 scopus 로고    scopus 로고
    • Assembly of the dystrophin-associated protein complex does not require the dystrophin COOH-terminal domain
    • Crawford, GE, Faulkner, JA, Crosbie, RH, Campbell, KP, Froehner, SC and Chamberlain, JS (2000). Assembly of the dystrophin-associated protein complex does not require the dystrophin COOH-terminal domain. J Cell Biol 150: 1399-1410.
    • (2000) J Cell Biol , vol.150 , pp. 1399-1410
    • Crawford, G.E.1    Faulkner, J.A.2    Crosbie, R.H.3    Campbell, K.P.4    Froehner, S.C.5    Chamberlain, J.S.6
  • 7
    • 33745191588 scopus 로고    scopus 로고
    • A highly functional mini-dystrophin/GFP fusion gene for cell and gene therapy studies of Duchenne muscular dystrophy
    • Li, S, Kimura, E, Ng, R, Fall, BM, Meuse, L, Reyes, M et al. (2006). A highly functional mini-dystrophin/GFP fusion gene for cell and gene therapy studies of Duchenne muscular dystrophy. Hum Mol Genet 15: 1610-1622.
    • (2006) Hum Mol Genet , vol.15 , pp. 1610-1622
    • Li, S.1    Kimura, E.2    Ng, R.3    Fall, B.M.4    Meuse, L.5    Reyes, M.6
  • 8
    • 33745248418 scopus 로고    scopus 로고
    • C-terminal truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double knock-out mice
    • Yue, Y, Liu, M and Duan, D (2006). C-terminal truncated microdystrophin recruits dystrobrevin and syntrophin to the dystrophin-associated glycoprotein complex and reduces muscular dystrophy in symptomatic utrophin/dystrophin double knock-out mice. Mol Ther 14: 79-87.
    • (2006) Mol Ther , vol.14 , pp. 79-87
    • Yue, Y.1    Liu, M.2    Duan, D.3
  • 9
    • 30344435305 scopus 로고    scopus 로고
    • Gene therapy strategies for Duchenne muscular dystrophy utilizing recombinant adeno-associated viral vectors
    • Blankinship, MJ, Gregorevic, P and Chamberlain, JS (2006). Gene therapy strategies for Duchenne muscular dystrophy utilizing recombinant adeno-associated viral vectors. Mol Ther 13: 241-249.
    • (2006) Mol Ther , vol.13 , pp. 241-249
    • Blankinship, M.J.1    Gregorevic, P.2    Chamberlain, J.S.3
  • 10
    • 33845218878 scopus 로고    scopus 로고
    • Gene therapy progress and prospects: Duchenne muscular dystrophy
    • Foster, K, Foster, H and Dickson, JG (2006). Gene therapy progress and prospects: Duchenne muscular dystrophy. Gene Ther 13: 1677-1685.
    • (2006) Gene Ther , vol.13 , pp. 1677-1685
    • Foster, K.1    Foster, H.2    Dickson, J.G.3
  • 12
    • 0036127393 scopus 로고    scopus 로고
    • Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy
    • Harper, SQ, Hauser, MA, DelloRusso, C, Duan, D, Crawford, RW, Phelps, SF et al. (2002). Modular flexibility of dystrophin: Implications for gene therapy of Duchenne muscular dystrophy. Nat Med 8: 253-261.
    • (2002) Nat Med , vol.8 , pp. 253-261
    • Harper, S.Q.1    Hauser, M.A.2    DelloRusso, C.3    Duan, D.4    Crawford, R.W.5    Phelps, S.F.6
  • 13
    • 0037350193 scopus 로고    scopus 로고
    • Nkx2.5 homeoprotein regulates expression of gap junction protein connexin 43 and sarcomere organization in postnatal cardiomyocytes
    • Kasahara, H, Ueyama, T, Wakimoto, H, Liu, MK, Maguire, CT, Converso, KL et al. (2003). Nkx2.5 homeoprotein regulates expression of gap junction protein connexin 43 and sarcomere organization in postnatal cardiomyocytes. J Mol Cell Cardiol 35: 243-256.
    • (2003) J Mol Cell Cardiol , vol.35 , pp. 243-256
    • Kasahara, H.1    Ueyama, T.2    Wakimoto, H.3    Liu, M.K.4    Maguire, C.T.5    Converso, K.L.6
  • 14
    • 4644288506 scopus 로고    scopus 로고
    • Disruption of Rho signaling results in progressive atrioventricular conduction defects while ventricular function remains preserved
    • Wei, L, Taffet, GE, Khoury, DS, Bo, J, Li, Y, Yatani, A et al. (2004). Disruption of Rho signaling results in progressive atrioventricular conduction defects while ventricular function remains preserved. FASEB J 18: 857-859.
    • (2004) FASEB J , vol.18 , pp. 857-859
    • Wei, L.1    Taffet, G.E.2    Khoury, D.S.3    Bo, J.4    Li, Y.5    Yatani, A.6
  • 15
    • 48749127844 scopus 로고    scopus 로고
    • Coxsackievirus and adenovirus receptor (CAR) mediates atrioventricular-node function and connexin 45 localization in the murine heart
    • Lim, BK, Xiong, D, Dorner, A, Youn, TJ, Yung, A, Liu, TI et al. (2008). Coxsackievirus and adenovirus receptor (CAR) mediates atrioventricular-node function and connexin 45 localization in the murine heart. J Clin Invest 118: 2758-2770.
    • (2008) J Clin Invest , vol.118 , pp. 2758-2770
    • Lim, B.K.1    Xiong, D.2    Dorner, A.3    Youn, T.J.4    Yung, A.5    Liu, T.I.6
  • 16
    • 0026342911 scopus 로고
    • Tissue-specific regulation of the alpha-myosin heavy chain gene promoter in transgenic mice
    • Subramaniam, A, Jones, WK, Gulick, J, Wert, S, Neumann, J and Robbins, J (1991). Tissue-specific regulation of the alpha-myosin heavy chain gene promoter in transgenic mice. J Biol Chem 266: 24613-24620.
    • (1991) J Biol Chem , vol.266 , pp. 24613-24620
    • Subramaniam, A.1    Jones, W.K.2    Gulick, J.3    Wert, S.4    Neumann, J.5    Robbins, J.6
  • 17
    • 37849000464 scopus 로고    scopus 로고
    • Prevention of dystrophin-deficient cardiomyopathy in twenty-one-month-old carrier mice by mosaic dystrophin expression or complementary dystrophin/utrophin expression
    • Bostick, B, Yue, Y, Long, C and Duan, D (2008). Prevention of dystrophin-deficient cardiomyopathy in twenty-one-month-old carrier mice by mosaic dystrophin expression or complementary dystrophin/utrophin expression. Circ Res 102: 121-130.
    • (2008) Circ Res , vol.102 , pp. 121-130
    • Bostick, B.1    Yue, Y.2    Long, C.3    Duan, D.4
  • 18
    • 50649083054 scopus 로고    scopus 로고
    • AAV-9 micro-dystrophin gene therapy ameliorates electrocardiographic abnormalities in mdx mice
    • Bostick, B, Yue, Y, Lai, Y, Long, C, Li, D and Dongsheng, D (2008). AAV-9 micro-dystrophin gene therapy ameliorates electrocardiographic abnormalities in mdx mice. Hum Gene Ther 19: 851-856.
    • (2008) Hum Gene Ther , vol.19 , pp. 851-856
    • Bostick, B.1    Yue, Y.2    Lai, Y.3    Long, C.4    Li, D.5    Dongsheng, D.6
  • 19
    • 0035403114 scopus 로고    scopus 로고
    • Dystrophin-deficient cardiomyocytes are abnormally vulnerable to mechanical stress-induced contractile failure and injury
    • Danialou, G, Comtois, AS, Dudley, R, Karpati, G, Vincent, G, Des Rosiers, C et al. (2001). Dystrophin-deficient cardiomyocytes are abnormally vulnerable to mechanical stress-induced contractile failure and injury. FASEB J 15: 1655-1657.
    • (2001) FASEB J , vol.15 , pp. 1655-1657
    • Danialou, G.1    Comtois, A.S.2    Dudley, R.3    Karpati, G.4    Vincent, G.5    Des Rosiers, C.6
  • 20
    • 0037711391 scopus 로고    scopus 로고
    • Exercise assessment of transgenic models of human cardiovascular disease
    • Bernstein, D (2003). Exercise assessment of transgenic models of human cardiovascular disease. Physiol Genomics 13: 217-226.
    • (2003) Physiol Genomics , vol.13 , pp. 217-226
    • Bernstein, D.1
  • 21
    • 0141841602 scopus 로고    scopus 로고
    • Microdystrophin gene therapy of cardiomyopathy restores dystrophin-glycoprotein complex and improves sarcolemma integrity in the mdx mouse heart
    • Yue, Y, Li, Z, Harper, SQ, Davisson, RL, Chamberlain, JS and Duan, D (2003). Microdystrophin gene therapy of cardiomyopathy restores dystrophin-glycoprotein complex and improves sarcolemma integrity in the mdx mouse heart. Circulation 108: 1626-1632.
    • (2003) Circulation , vol.108 , pp. 1626-1632
    • Yue, Y.1    Li, Z.2    Harper, S.Q.3    Davisson, R.L.4    Chamberlain, J.S.5    Duan, D.6
  • 22
    • 34249337407 scopus 로고    scopus 로고
    • Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure
    • Townsend, D, Blankinship, MJ, Allen, JM, Gregorevic, P, Chamberlain, JS and Metzger, JM (2007). Systemic administration of micro-dystrophin restores cardiac geometry and prevents dobutamine-induced cardiac pump failure. Mol Ther 15: 1086-1092.
    • (2007) Mol Ther , vol.15 , pp. 1086-1092
    • Townsend, D.1    Blankinship, M.J.2    Allen, J.M.3    Gregorevic, P.4    Chamberlain, J.S.5    Metzger, J.M.6
  • 23
    • 12744269885 scopus 로고    scopus 로고
    • Adeno-associated virus-mediated micro-dystrophin expression protects young mdx muscle from contraction-induced injury
    • Liu, M, Yue, Y, Harper, SQ, Grange, RW, Chamberlain, JS and Duan, D (2005). Adeno-associated virus-mediated micro-dystrophin expression protects young mdx muscle from contraction-induced injury. Mol Ther 11: 245-256.
    • (2005) Mol Ther , vol.11 , pp. 245-256
    • Liu, M.1    Yue, Y.2    Harper, S.Q.3    Grange, R.W.4    Chamberlain, J.S.5    Duan, D.6
  • 24
    • 27944479711 scopus 로고    scopus 로고
    • Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors
    • Lai, Y, Yue, Y, Liu, M, Ghosh, A, Engelhardt, JF, Chamberlain, JS et al. (2005). Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors. Nat Biotechnol 23: 1435-1439.
    • (2005) Nat Biotechnol , vol.23 , pp. 1435-1439
    • Lai, Y.1    Yue, Y.2    Liu, M.3    Ghosh, A.4    Engelhardt, J.F.5    Chamberlain, J.S.6
  • 25
    • 0033017374 scopus 로고    scopus 로고
    • Enteroviral protease 2A cleaves dystrophin: Evidence of cytoskeletal disruption in an acquired cardiomyopathy
    • Badorff, C, Lee, GH, Lamphear, BJ, Martone, ME, Campbell, KP, Rhoads, RE et al. (1999). Enteroviral protease 2A cleaves dystrophin: evidence of cytoskeletal disruption in an acquired cardiomyopathy. Nat Med 5: 320-326.
    • (1999) Nat Med , vol.5 , pp. 320-326
    • Badorff, C.1    Lee, G.H.2    Lamphear, B.J.3    Martone, M.E.4    Campbell, K.P.5    Rhoads, R.E.6
  • 26
    • 0037211804 scopus 로고    scopus 로고
    • Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle
    • Wells, KE, Torelli, S, Lu, Q, Brown, SC, Partridge, T, Muntoni, F et al. (2003). Relocalization of neuronal nitric oxide synthase (nNOS) as a marker for complete restoration of the dystrophin associated protein complex in skeletal muscle. Neuromuscul Disord 13: 21-31.
    • (2003) Neuromuscul Disord , vol.13 , pp. 21-31
    • Wells, K.E.1    Torelli, S.2    Lu, Q.3    Brown, S.C.4    Partridge, T.5    Muntoni, F.6
  • 27
    • 7044223308 scopus 로고    scopus 로고
    • Absence of neuronal nitric oxide synthase (nNOS) as a pathological marker for the diagnosis of Becker muscular dystrophy with rod domain deletions
    • Torelli, S, Brown, SC, Jimenez-Mallebrera, C, Feng, L, Muntoni, F and Sewry, CA (2004). Absence of neuronal nitric oxide synthase (nNOS) as a pathological marker for the diagnosis of Becker muscular dystrophy with rod domain deletions. Neuropathol Appl Neurobiol 30: 540-545.
    • (2004) Neuropathol Appl Neurobiol , vol.30 , pp. 540-545
    • Torelli, S.1    Brown, S.C.2    Jimenez-Mallebrera, C.3    Feng, L.4    Muntoni, F.5    Sewry, C.A.6
  • 28
    • 33646675371 scopus 로고    scopus 로고
    • Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex
    • Judge, LM, Haraguchi, M and Chamberlain, JS (2006). Dissecting the signaling and mechanical functions of the dystrophin-glycoprotein complex. J Cell Sci 119: 1537-1546.
    • (2006) J Cell Sci , vol.119 , pp. 1537-1546
    • Judge, L.M.1    Haraguchi, M.2    Chamberlain, J.S.3
  • 29
    • 0032411144 scopus 로고    scopus 로고
    • Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle
    • Thomas, GD, Sander, M, Lau, KS, Huang, PL, Stull, JT and Victor, RG (1998). Impaired metabolic modulation of alpha-adrenergic vasoconstriction in dystrophin-deficient skeletal muscle. Proc Natl Acad Sci USA 95: 15090-15095.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 15090-15095
    • Thomas, G.D.1    Sander, M.2    Lau, K.S.3    Huang, P.L.4    Stull, J.T.5    Victor, R.G.6
  • 30
    • 0032735294 scopus 로고    scopus 로고
    • Cardiomyopathy in Duchenne, Becker, and sarcoglycanopathies: A role for coronary dysfunction?
    • Gnecchi-Ruscone, T, Taylor, J, Mercuri, E, Paternostro, G, Pogue, R, Bushby, K et al. (1999). Cardiomyopathy in Duchenne, Becker, and sarcoglycanopathies: A role for coronary dysfunction? Muscle Nerve 22: 1549-1556.
    • (1999) Muscle Nerve , vol.22 , pp. 1549-1556
    • Gnecchi-Ruscone, T.1    Taylor, J.2    Mercuri, E.3    Paternostro, G.4    Pogue, R.5    Bushby, K.6
  • 31
    • 0042522638 scopus 로고    scopus 로고
    • Myocardial cell damage in Duchenne muscular dystrophy
    • Ramaciotti, C, Iannaccone, ST and Scott, WA (2003). Myocardial cell damage in Duchenne muscular dystrophy. Pediatr Cardiol 24: 503-506.
    • (2003) Pediatr Cardiol , vol.24 , pp. 503-506
    • Ramaciotti, C.1    Iannaccone, S.T.2    Scott, W.A.3
  • 35
    • 3442885991 scopus 로고    scopus 로고
    • Multiple pathogenetic mechanisms in X linked dilated cardiomyopathy
    • Cohen, N and Muntoni, F (2004). Multiple pathogenetic mechanisms in X linked dilated cardiomyopathy. Heart 90: 835-841.
    • (2004) Heart , vol.90 , pp. 835-841
    • Cohen, N.1    Muntoni, F.2
  • 36
    • 0027193330 scopus 로고
    • X-linked dilated cardiomyopathy. Molecular genetic evidence of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus
    • Towbin, JA, Hejtmancik, JF, Brink, P, Gelb, B, Zhu, XM, Chamberlain, JS et al. (1993). X-linked dilated cardiomyopathy. Molecular genetic evidence of linkage to the Duchenne muscular dystrophy (dystrophin) gene at the Xp21 locus. Circulation 87: 1854-1865.
    • (1993) Circulation , vol.87 , pp. 1854-1865
    • Towbin, J.A.1    Hejtmancik, J.F.2    Brink, P.3    Gelb, B.4    Zhu, X.M.5    Chamberlain, J.S.6
  • 38
    • 0020448805 scopus 로고
    • Cardiac function in Duchenne's muscular dystrophy. Results of 10-year follow-up study and noninvasive tests
    • Hunsaker, RH, Fulkerson, PK, Barry, FJ, Lewis, RP, Leier, CV and Unverferth, DV (1982). Cardiac function in Duchenne's muscular dystrophy. Results of 10-year follow-up study and noninvasive tests. Am J Med 73: 235-238.
    • (1982) Am J Med , vol.73 , pp. 235-238
    • Hunsaker, R.H.1    Fulkerson, P.K.2    Barry, F.J.3    Lewis, R.P.4    Leier, C.V.5    Unverferth, D.V.6
  • 39
    • 0027256794 scopus 로고
    • Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy
    • Takenaka, A, Yokota, M, Iwase, M, Miyaguchi, K, Hayashi, H and Saito, H (1993). Discrepancy between systolic and diastolic dysfunction of the left ventricle in patients with Duchenne muscular dystrophy. Eur Heart J 14: 669-676.
    • (1993) Eur Heart J , vol.14 , pp. 669-676
    • Takenaka, A.1    Yokota, M.2    Iwase, M.3    Miyaguchi, K.4    Hayashi, H.5    Saito, H.6
  • 42
    • 0032693757 scopus 로고    scopus 로고
    • Submucosal gland development in the airway is controlled by lymphoid enhancer binding factor 1 (LEF1)
    • Duan, D, Yue, Y, Zhou, W, Labed, B, Ritche, TC, Grosschedl, R et al. (1999). Submucosal gland development in the airway is controlled by lymphoid enhancer binding factor 1 (LEF1). Development 126: 4441-4453.
    • (1999) Development , vol.126 , pp. 4441-4453
    • Duan, D.1    Yue, Y.2    Zhou, W.3    Labed, B.4    Ritche, T.C.5    Grosschedl, R.6
  • 43
    • 4344672569 scopus 로고    scopus 로고
    • Full-length dystrophin expression in half of the heart cells ameliorates beta-isoproterenol-induced cardiomyopathy in mdx mice
    • Yue, Y, Skimming, JW, Liu, M, Strawn, T and Duan, D (2004). Full-length dystrophin expression in half of the heart cells ameliorates beta-isoproterenol-induced cardiomyopathy in mdx mice. Hum Mol Genet 13: 1669-1675.
    • (2004) Hum Mol Genet , vol.13 , pp. 1669-1675
    • Yue, Y.1    Skimming, J.W.2    Liu, M.3    Strawn, T.4    Duan, D.5
  • 44
    • 0014154751 scopus 로고
    • Determination of hydroxyproline
    • Stegemann, H and Stalder, K (1967). Determination of hydroxyproline. Clin Chim Acta 18: 267-273.
    • (1967) Clin Chim Acta , vol.18 , pp. 267-273
    • Stegemann, H.1    Stalder, K.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.