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Stress Protein Shows Promise in Lou Gehrig's Disease

Study shows increased life span, delayed disease progression in mouse model of amyotrophic lateral sclerosis

THURSDAY, Nov. 29 (HealthDay News) -- Injecting a mouse model of amyotrophic lateral sclerosis with a protein that protects cells from stress increases their life span and delays disease progression, according to research published in the Nov. 28 issue of the Journal of Neuroscience.

David J. Gifondorwa, and colleagues from Wake Forest University School of Medicine in Winston-Salem, N.C., injected recombinant human Hsp70 (rhHsp70) into G93A mutant SOD1 mice, a model of amyotrophic lateral sclerosis, three times a week from postnatal day 50 until end-stage. A lack of Hsp70 upregulation after stress has been implicated in the death of motor neurons, they note.

The researchers found that rhHsp70 increased life span, delayed symptom onset, preserved motor function, increased the survival of motor neurons, and increased the number of innervated neuromuscular junctions. The injected rhHsp70 was found in skeletal muscle and not detected in the central nervous system.

"Together these results suggest rhHsp70 may delay disease progression in the G93A SOD1 mouse via a yet to be identified peripheral mechanism," Gifondorwa and colleagues conclude.

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