Autophagy in Huntington disease and huntingtin in autophagy Oct 1st 2014, 00:00, by Dale D.O. Martin, Safia Ladha, Dagmar E. Ehrnhoefer, Michael R. Hayden HD is a devastating neurological disease that is characterized by loss of motor control and cognitive ability and, ultimately, death. The area of the brain most affected by the disease is the striatum, which plays a key role in initiating and controlling movements of the body, limbs and eyes [1]. HD is an autosomal dominant disease caused by a CAG expansion that encodes a polyQ repeat at the N-terminus of HTT [2]. Many factors have been implicated in HD including alterations in calcium handling, IGF signaling, vesicle transport, endoplasmic reticulum (ER) maintenance, and autophagy. |