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Clues to the Mystery of Lou Gehrig's Disease

entia and motor neuron disease. Clinically there's overlap in these two disorders, so it was very tantalizing to see if there was anything to link them biochemically."

The researchers developed monoclonal antibodies to TDP-43, and tested them in 72 post-mortem tissue samples patients with either ALS or frontotemporal lobar degeneration and found that all 72 cases contained misfolded TDP-43. Dr Lee said, "Since many cases were studied, the data became very compelling."

According to study co-author John Trojanowksi, M.D., Ph.D., director of Penn's Institute on Aging although ALS is well-known because of its link with Gehrig, frontotemporal lobar degeneration is less well-known while this may be because of its rarity in spite of it being the second most common cause -- after Alzheimer's disease -- of dementia in people under the age of 65.

Dr. Trojanowksi said, "Another reason for (its) relative obscurity is also undoubtedly the shame family members experience with the strange and, at times, bizarre behavior of patients, including disturbing obsessions, larceny, or even sexual deviancy, that may bring the patient to the attention of the legal system rather than to the healthcare system."

Dr Lee pointed out that usefulness of the finding depends on whether future research shows that TDP-43 is a useful target for new therapies and diagnostic tools.
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