Fast skeletal muscle troponin activator tirasemtiv increases muscle function and performance in the B6SJL-SOD1G93A ALS mouse model

PLoS ONE

Volumn 9, Issue 5, 2014, Pages

  Hwee, Darren T ^a   Kennedy, Adam ^a   Ryans, Julie ^a   Russell, Alan J ^a   Jia, Zhiheng ^a   Hinken, Aaron C ^a   Morgans, David J ^a   Malik, Fady I ^a   Jasper, Jeffrey R ^a  

^a Cytokinetics Inc   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

TIRASEMTIV; TROPONIN; CK-2017357; IMIDAZOLE DERIVATIVE; PYRAZINE DERIVATIVE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; COHORT ANALYSIS; CONTROLLED STUDY; DIAPHRAGM MUSCLE; DOSE RESPONSE; FEMALE; FORELIMB; GRIP STRENGTH; MOTOR DYSFUNCTION; MOTOR PERFORMANCE; MOUSE; MUSCLE FUNCTION; MUSCLE ISOMETRIC CONTRACTION; MUSCLE STRENGTH; NONHUMAN; SINGLE DRUG DOSE; SKELETAL MUSCLE; TIDAL VOLUME; TRANSGENIC MOUSE; TREATMENT RESPONSE; ANIMAL; DISEASE MODEL; DRUG EFFECTS; GENETICS; HUMAN; METABOLISM; MOTONEURON; PATHOLOGY;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; DISEASE MODELS, ANIMAL; FEMALE; HUMANS; IMIDAZOLES; MICE; MICE, TRANSGENIC; MOTOR NEURONS; MUSCLE STRENGTH; MUSCLE, SKELETAL; PYRAZINES; TROPONIN;

EID: 84900563424     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0096921     Document Type: Article

References (25)

1. Logroscino G, Traynor BJ, Hardiman O, Chio A, Mitchell D, et al. (2010) Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 81: 385-390.
2. Bruijn LI, Miller TM, Cleveland DW (2004) Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci 27: 723-749. (Pubitemid 39050419)
3. Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, et al. (1994) Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation. Science 264: 1772-1775. (Pubitemid 24227760)
4. Hegedus J, Putman CT, Gordon T (2007) Time course of preferential motor unit loss in the SOD1 G93A mouse model of amyotrophic lateral sclerosis. Neurobiol Dis 28: 154-164. (Pubitemid 47615455)
5. Gurney ME, Cutting FB, Zhai P, Doble A, Taylor CP, et al. (1996) Benefit of vitamin E, riluzole, and gabapentin in a transgenic model of familial amyotrophic lateral sclerosis. Ann Neurol 39: 147-157. (Pubitemid 26071564)
6. (2012) Rilutek Package Insert. sanofi-aventis.
7. Miller RG, Mitchell JD, Lyon M, Moore DH (2003) Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Amyotroph Lateral Scler Other Motor Neuron Disord 4: 191-206. (Pubitemid 37184890)
8. Russell AJ, Hartman JJ, Hinken AC, Muci AR, Kawas R, et al. (2012) Activation of fast skeletal muscle troponin as a potential therapeutic approach for treating neuromuscular diseases. Nat Med 18: 452-455.
9. Acevedo-Arozena A, Kalmar B, Essa S, Ricketts T, Joyce P, et al. (2011) A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis. Dis Model Mech 4: 686-700.
10. Smittkamp SE, Brown JW, Stanford JA (2008) Time-course and characterization of orolingual motor deficits in B6SJL-Tg(SOD1-G93A)1Gur/J mice. Neuroscience 151: 613-621.
11. Papadeas ST, Kraig SE, O'Banion C, Lepore AC, Maragakis NJ (2011) Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo. Proc Natl Acad Sci U S A 108: 17803-17808.
12. Siirala W, Saaresranta T, Vuori A, Salantera S, Olkkola KT, et al. (2012) Using respiratory rate and thoracic movement to assess respiratory insufficiency in amyotrophic lateral sclerosis: a preliminary study. BMC Palliat Care 11: 26.
13. Vitacca M, Clini E, Facchetti D, Pagani M, Poloni M, et al. (1997) Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis. Eur Respir J 10: 1614-1621. (Pubitemid 27311081)
14. Llado J, Haenggeli C, Pardo A,Wong V, Benson L, et al. (2006) Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS. Neurobiol Dis 21: 110-118. (Pubitemid 41817311)
15. Miciak JJ, Warsing LC, Tibbs ME, Jasper JR, Jampel SB, et al. (2013) A fast skeletal muscle troponin activator in the dy2J muscular dystrophy model. Muscle Nerve.
16. Schnabel J (2008) Neuroscience: Standard model. Nature 454: 682-685.
17. Shefner J, Cedarbaum JM, Cudkowicz ME, Maragakis N, Lee J, et al. (2012) Safety, tolerability and pharmacodynamics of a skeletal muscle activator in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 13: 430-438.
18. Wijesekera LC, Leigh PN (2009) Amyotrophic lateral sclerosis. Orphanet J Rare Dis 4: 3.
19. Silani V, Messina S, Poletti B, Morelli C, Doretti A, et al. (2011) The diagnosis of Amyotrophic lateral sclerosis in 2010. Arch Ital Biol 149: 5-27.
20. Tankersley CG HC, Rothstein JD (2007) Respiratory impairment in a mouse model of amyotrophic lateral sclerosis. J Appl Physiol 102: 926-932.
21. Morrison BM, Lachey JL, Warsing LC, Ting BL, Pullen AE, et al. (2009) A soluble activin type IIB receptor improves function in a mouse model of amyotrophic lateral sclerosis. Exp Neurol 217: 258-268.
22. Da Cruz S, Parone PA, Lopes VS, Lillo C, McAlonis-Downes M, et al. (2012) Elevated PGC-1alpha activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Cell Metab 15: 778-786.
23. Dobrowolny G, Giacinti C, Pelosi L, Nicoletti C, Winn N, et al. (2005) Muscle expression of a local Igf-1 isoform protects motor neurons in an ALS mouse model. J Cell Biol 168: 193-199. (Pubitemid 40248217)
24. Sorenson EJ, Windbank AJ, Mandrekar JN, Bamlet WR, Appel SH, et al. (2008) Subcutaneous IGF-1 is not beneficial in 2-year ALS trial. Neurology 71: 1770-1775.
25. Jasmin BJ (1987) Patterns of EMG activity of rat plantaris muscle during swimming and other locomotor activities. J Appl Physiol 63.