Functional and molecular effects of arginine butyrate and prednisone on muscle and heart in the mdx mouse model of duchenne muscular dystrophy

PLoS ONE

Volumn 5, Issue 6, 2010, Pages

  Guerron, Alfredo D ^a   Rawat, Rashmi ^a   Sali, Arpana ^a   Spurney, Christopher F ^b   Pistilli, Emidio ^c   Cha, Hee Jae ^d   Pandey, Gouri S ^a   Gernapudi, Ramkishore ^a   Francia, Dwight ^a   Farajian, Viken ^a   Escolar, Diana M ^a   Bossi, Laura ^e   Becker, Magali ^e   Zerr, Patricia ^e   de la Porte, Sabine ^f   Gordish Dressman, Heather ^a   Partridge, Terence ^a   Hoffman, Eric P ^a   Nagaraju, Kanneboyina ^a  

^a GEORGE WASHINGTON UNIVERSITY SCHOOL OF MEDICINE AND HEALTH SCIENCES   (United States)

^b CHILDREN'S NATIONAL MEDICAL CENTER   (United States)

^c UNIVERSITY OF PENNSYLVANIA   (United States)

^d Kosin University College of Medicine   (South Korea)

^e TRANSGENE S A   (France)

^f Institut Fédé ratif de Neurobiologie Alfred Fessard   (France)

Author keywords

[No Author keywords available]

Indexed keywords

ARGININE BUTYRATE; BUTYRIC ACID DERIVATIVE; CREATININE; HISTONE DEACETYLASE; PREDNISONE; UNCLASSIFIED DRUG; ARGININE; DRUG DERIVATIVE; MESSENGER RNA; UTROPHIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR; CELL DIFFERENTIATION; CELL GROWTH; CELL PROLIFERATION; CONTROLLED STUDY; CREATININE BLOOD LEVEL; DRUG ACTIVITY; DRUG DETERMINATION; DRUG EFFECT; DRUG MECHANISM; DRUG MONITORING; DUCHENNE MUSCULAR DYSTROPHY; ENZYME ACTIVITY; GASTROCNEMIUS MUSCLE; GENE EXPRESSION; GRIP STRENGTH; HEART FUNCTION; IN VIVO STUDY; INFLAMMATION; LONG TERM CARE; MOUSE; MUSCLE ATROPHY; MUSCLE DEVELOPMENT; MUSCLE FUNCTION; MYOFIBROSIS; NONHUMAN; TREATMENT DURATION; X CHROMOSOME LINKED MUSCULAR DYSTROPHIC MOUSE; ANIMAL; ANIMAL BEHAVIOR; DISEASE MODEL; DRUG COMBINATION; FEMALE; GENE EXPRESSION PROFILING; GENE EXPRESSION REGULATION; GENETICS; HEART; METABOLISM; MUSCLE; PATHOLOGY; PATHOPHYSIOLOGY; TIME;

MUS;

ANIMALS; ARGININE; BEHAVIOR, ANIMAL; BUTYRATES; DISEASE MODELS, ANIMAL; DRUG THERAPY, COMBINATION; FEMALE; GENE EXPRESSION PROFILING; GENE EXPRESSION REGULATION; HEART; MICE; MICE, INBRED MDX; MUSCLES; MUSCULAR DYSTROPHY, DUCHENNE; PREDNISONE; RNA, MESSENGER; TIME FACTORS; UTROPHIN;

EID: 77955288979     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0011220     Document Type: Article

References (30)

1. Spurney CF, Gordish-Dressman H, Guerron AD, Sali A, Pandey GS, et al. (2009) Preclinical drug trials in the mdx mouse: assessment of reliable and sensitive outcome measures. Muscle Nerve 39: 591-602.
2. Raben N, Nagaraju K, Lee E, Plotz P (2000) Modulation of disease severity in mice with targeted disruption of the acid alpha-glucosidase gene. Neuromuscul Disord 10: 283-291.
3. Nagaraju K, Casciola-Rosen L, Rosen A, Thompson C, Loeffler L, et al. (2000) The inhibition of apoptosis in myositis and in normal muscle cells. J Immunol 164: 5459-5465.
4. Spurney CF, Knoblach S, Pistilli EE, Nagaraju K, Martin GR, et al. (2008) Dystrophin-deficient cardiomyopathy in mouse: Expression of Nox4 and Lox are associated with fibrosis and altered functional parameters in the heart. Neuromuscul Disord 18: 371-381.
5. Lamason RL, Mohideen MA, Mest JR, Wong AC, Norton HL, et al. (2005) SLC24A5, a putative cation exchanger, affects pigmentation in zebrafish and humans. Science 310: 1782-1786.
6. Candido EP, Reeves R, Davie JR (1978) Sodium butyrate inhibits histone deacetylation in cultured cells. Cell 14: 105-113.
7. Kumar-Singh R, Chamberlain JS (1996) Encapsidated adenovirus minichromosomes allow delivery and expression of a 14 kb dystrophin cDNA to muscle cells. Hum Mol Genet 5: 913-921.
8. Anderson JE, Weber M, Vargas C (2000) Deflazacort increases laminin expression and myogenic repair, and induces early persistent functional gain in mdx mouse muscular dystrophy. Cell Transplant 9: 551-564.
9. Anderson JE, McIntosh LM, Poettcker R (1996) Deflazacort but not prednisone improves both muscle repair and fiber growth in diaphragm and limb muscle in vivo in the mdx dystrophic mouse. Muscle Nerve 19: 1576-1585.
10. Hudecki MS, Pollina CM, Granchelli JA, Daly MK, Byrnes T, et al. (1993) Strength and endurance in the therapeutic evaluation of prednisolone-treated MDX mice. Res Commun Chem Pathol Pharmacol 79: 45-60.
11. Granchelli JA, Pollina C, Hudecki MS (2000) Pre-clinical screening of drugs using the mdx mouse. Neuromuscul Disord 10: 235-239.
12. Granchelli JA, Avosso DL, Hudecki MS, Pollina C (1996) Cromolyn increases strength in exercised mdx mice. Res Commun Mol Pathol Pharmacol 91: 287-296.
13. De Luca A, Pierno S, Liantonio A, Cetrone M, Camerino C, et al. (2003) Enhanced dystrophic progression in mdx mice by exercise and beneficial effects of taurine and insulin-like growth factor-1. J Pharmacol Exp Ther 304: 453-463.
14. Hartel JV, Granchelli JA, Hudecki MS, Pollina CM, Gosselin LE (2001) Impact of prednisone on TGF-beta1 and collagen in diaphragm muscle from mdx mice. Muscle Nerve 24: 428-432.
15. Connolly AM, Schierbecker J, Renna R, Florence J (2002) High dose weekly oral prednisone improves strength in boys with Duchenne muscular dystrophy. Neuromuscul Disord 12: 917-925.
16. Keeling RM, Golumbek PT, Streif EM, Connolly AM (2007) Weekly oral prednisolone improves survival and strength in male mdx mice. Muscle Nerve 35: 43-48.
17. Yang L, Luo J, Petrof BJ (1998) Corticosteroid therapy does not alter the threshold for contraction-induced injury in dystrophic (mdx) mouse diaphragm. Muscle Nerve 21: 394-397.
18. De Luca A, Pierno S, Liantonio A, Conte Camerino D (2002) Pre-clinical trials in Duchenne dystrophy: what animal models can tell us about potential drug effectiveness. Neuromuscul Disord 12: Suppl 1: S142-146.
19. Sweat F, Puchtler H, Rosenthal SI (1964) Sirius Red F3ba as a Stain for Connective Tissue. Arch Pathol 78: 69-72.
20. Hoey AJ, Erp CV (2006) Effect of L-arginine on cardiac function and fibrosis in mdx mice. Proceedings of the Australian Physiological Society 37: 57.
21. Hnia K, Gayraud J, Hugon G, Ramonatxo M, De La Porte S, et al. (2008) L-arginine decreases inflammation and modulates the nuclear factor-kappaB/matrix metalloproteinase cascade in mdx muscle fibers. Am J Pathol 172: 1509-1519.
22. Reyes AA, Karl IE, Klahr S (1994) Role of arginine in health and in renal disease. Am J Physiol 267: F331-346.
23. Morrissey JJ, Ishidoya S, McCracken R, Klahr S (1996) Nitric oxide generation ameliorates the tubulointerstitial fibrosis of obstructive nephropathy. J Am Soc Nephrol 7: 2202-2212.
24. Archer JD, Vargas CC, Anderson JE (2006) Persistent and improved functional gain in mdx dystrophic mice after treatment with L-arginine and deflazacort. Faseb J 20: 738-740.
25. Voisin V, Sebrie C, Matecki S, Yu H, Gillet B, et al. (2005) L-arginine improves dystrophic phenotype in mdx mice. Neurobiol Dis 20: 123-130.
26. Chazalette D, Hnia K, Rivier F, Hugon G, Mornet D (2005) alpha7B integrin changes in mdx mouse muscles after L-arginine administration. FEBS Lett 579: 1079-1084.
27. Barton ER, Morris L, Kawana M, Bish LT, Toursel T (2005) Systemic administration of L-arginine benefits mdx skeletal muscle function. Muscle Nerve 32: 751-760.
28. St-Pierre SJ, Chakkalakal JV, Kolodziejczyk SM, Knudson JC, Jasmin BJ, et al. (2004) Glucocorticoid treatment alleviates dystrophic myofiber pathology by activation of the calcineurin/NF-AT pathway. Faseb J 18: 1937-1939.
29. Miura P, Andrews M, Holcik M, Jasmin BJ (2008) IRES-mediated translation of utrophin A is enhanced by glucocorticoid treatment in skeletal muscle cells. PLoS ONE 3: e2309.
30. Khurana TS, Davies KE (2003) Pharmacological strategies for muscular dystrophy. Nat Rev Drug Discov 2: 379-390.