An American charity has given an Oxford gene therapy company £500,000 to investigate possible treatments for motor-neuron disease.

Oxford BioMedica, based at Oxford Science Park, has been given the grant from the charity Andrew's Buddies/FightSMA, which is dedicated to the development of treatments for Spinal Muscular Atrophy (SMA), a form of motor-neuron disease that is one of the most common causes of death in childhood.

The disease, caused by a defect in a gene known as SMN-1, affects one in every 6,000 births. One in 40 people are carriers.

It leads to a degeneration of motor-neurons in the spinal cord, causing loss of muscle function. Children with the most severe form of the disease usually die before the age of two, when their lungs stop working.

Oxford BioMedica will use genetic engineering to replace the defective gene.

The company will keep any commercial rights arising from the grant-funded programme and aims to produce a treatment which can be tested in hospital clinics within two years.

The researchers are also developing a treatment for the most common form of motor-neuron disease, amyotrophic lateral sclerosis.

At present there are no treatments for the devastating diseases.

The president of Andrew's Buddies/Fight SMA, Martha Slay, said: "We are delighted to fund this ground-breaking initiative.

"The work of Oxford BioMedica breathes new hope into the lives of countless parents and their children who suffer with SMA."

Oxford BioMedica's chief executive, Prof Alan Kingsman, said: "The development of novel products for neurological disease is the largest growth area in pharmaceutical research.

"Oxford BioMedica has an exceptional capability in this area and is developing a pipeline of products, including ProSavin for Parkinson's disease.

"We are delighted that Andrew's Buddies/Fight SMA has recognised the quality of our technology and we look forward to working together towards an effective treatment for this inherited disease that brings so much sorrow to afflicted families."