Therapeutic effects of clenbuterol in a murine model of amyotrophic lateral sclerosis.
We investigated the effects of clenbuterol, a beta2-adrenoceptor agonist with known anabolic and neuroprotective properties, on G93A-SOD1 mice, a transgenic murine model of familial amyotrophic lateral sclerosis (ALS). Relative to saline-treated vehicle controls (0.2 ml/kg/day; i.p.), early pathologic G93A-SOD1 mice treated with clenbuterol (1.5 mg/kg/day; i.p.) demonstrated a delayed onset of hindlimb signs as measured by rotarod performance, slowed disease progression, as well as trends toward mitigated losses of lumbar motoneurons and body weight. Responses in female G93A-SOD1 mice were favorable to those of males, suggesting synergistic effects between clenbuterol and sex-specific factors. Overall, our data suggest that clenbuterol offers therapeutic effects on ALS-related neuromuscular degeneration.
Teng
Yang D
YD
Department of Neurosurgery, Harvard Medical School/Children's Hospital Boston, MA, USA. yang_teng@hms.harvard.edu
Choi
Howard
H
Huang
Wenzheng
W
Onario
Renna C
RC
Frontera
Walter R
WR
Snyder
Evan Y
EY
Sabharwal
Sunil
S
D000318:Adrenergic beta-Agonists / Q000627:therapeutic use*; D000367:Age Factors; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000235:genetics / Q000401:mortality; D000818:Animals; D001835:Body Weight / Q000187:drug effects; D002976:Clenbuterol / Q000627:therapeutic use*; D004195:Disease Models, Animal; D005260:Female; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D009043:Motor Activity / Q000187:drug effects*; D011897:Random Allocation; D045442:Rotarod Performance Test / Q000379:methods; D012737:Sex Factors; D013482:Superoxide Dismutase / Q000235:genetics
D000318:Adrenergic beta-Agonists; C109415:SOD1 G93A protein; D013482:Superoxide Dismutase; D002976:Clenbuterol