T-588, a novel neuroprotective agent, delays progression of neuromuscular dysfunction in wobbler mouse motoneuron disease

Brain Res. 2000 Mar 6;858(1):84-91. doi: 10.1016/s0006-8993(99)02427-0.

Abstract

R(-)-1-(benzo[b]thiophen-5-yl)-2-[2-(N,N-diethylamino) ethoxy]ethanol hydrochloride (T-588) enhances acetylcholine release from the frontal cortex and hippocampus in rats, and can ameliorate cognitive dysfunction in various amnesia models of rodents. T-588 protects rat cerebellar granule cells from glutamate neurotoxicity in culture. This agent also inhibits facilitation in the crayfish neuromuscular junction and mammalian cerebellum. Clinical trials of T-588 are underway in patients with Alzheimer's disease. We attempted to determine whether T-588 treatment ameliorates neuromuscular dysfunction in the wobbler mouse, an animal model of motoneuron disease (MND). After the initial diagnosis of MND at the age of 3-4 weeks, wobbler mice were orally administered T-588 (3, 10, 30 mg/kg) or vehicle daily for 4 weeks in a blinded fashion. We compared symptomatic, pathological and biochemical changes among the groups. In comparison with vehicle, T-588 administration potentiated grip strength, attenuated forelimb contracture and increased the weight of the biceps muscles. T-588-treated mice had retarded denervation muscle atrophy and elevated activities of choline acetyltransferase (ChAT) or lactate dehydrogenase in the biceps muscles. T-588 treatment also enhanced ChAT activities and promoted formation of cyclic adenosine monophosphate in the cervical cord. Pharmacokinetic study also showed that T-588 was transported efficiently into the cerebrum and spinal cord following oral administration. Thus, T-588 treatment delayed the progression of wobbler murine MND. Our findings suggest that this agent has therapeutic potential in human motor neuropathy or MND.

MeSH terms

  • Administration, Oral
  • Animals
  • Cell Count / drug effects
  • Cells, Cultured
  • Central Nervous System Stimulants / administration & dosage*
  • Central Nervous System Stimulants / pharmacokinetics
  • Choline O-Acetyltransferase / metabolism
  • Cyclic AMP / metabolism
  • Diethylamines / administration & dosage*
  • Diethylamines / pharmacokinetics
  • Disease Models, Animal
  • Disease Progression
  • Drug Evaluation, Preclinical
  • Forelimb / pathology
  • Forelimb / physiopathology
  • Hand Strength / physiology
  • L-Lactate Dehydrogenase / metabolism
  • Mice
  • Mice, Inbred C57BL
  • Mice, Neurologic Mutants
  • Motor Neuron Disease / drug therapy*
  • Motor Neuron Disease / enzymology
  • Motor Neuron Disease / pathology
  • Muscle, Skeletal / drug effects
  • Muscle, Skeletal / enzymology
  • Muscle, Skeletal / pathology
  • Organ Size
  • Spinal Cord / drug effects
  • Spinal Cord / enzymology
  • Spinal Cord / pathology
  • Thiophenes / administration & dosage*
  • Thiophenes / pharmacokinetics

Substances

  • Central Nervous System Stimulants
  • Diethylamines
  • Thiophenes
  • T 588
  • Cyclic AMP
  • L-Lactate Dehydrogenase
  • Choline O-Acetyltransferase