Cause of Lou Gehrig disease discovered

CHICAGO, Aug. 22 (UPI) -- The cause of amyotrophic lateral sclerosis is a protein, whose job is to recycle damaged proteins in motor and cortical neurons, U.S. researchers say.

Dr. Teepu Siddique of Northwestern University's Feinberg School of Medicine and a neurologist at Northwestern Memorial Hospital who was the study's senior author says in ALS, also known as Lou Gehrig's disease, the protein ubiquilin2 isn't doing its job.

As a result, the damaged proteins and ubiquilin2 loiter and accumulate in the motor neurons in the spinal cord and cortical and hippocampal neurons in the brain resembling twisted skeins of yarn and cause the degeneration of the neurons.

The researchers found ubiquilin2 in these skein-like accumulations in the spinal cords of ALS cases and in the brains of ALS/dementia cases.

The scientists also discovered mutations in ubiquilin2 in patients with familial ALS and familial ALS/dementia. However, the skein-like accumulations were present in people's brains and spinal cords in all forms of ALS and ALS/dementia, whether or not they had the gene mutation, the study says.

The study, published in the journal Nature, provides a common target for drug therapy.