Detection of an Amadori product, 1-hexitol-lysine, in the anterior horn ofthe amyotrophic lateral sclerosis and spinobulbar muscular atrophy spinal cord: evidence for early involvement of glycation in motoneuron diseases

Glycation is a series of non-enzymatic reactions initiated by addition of r educing sugars to epsilon-amino group of lysine residues and cr-amino group of the N terminus of proteins, leading to the formation of advanced glycat ion end products (AGE), It is thought to be involved in aging and various n eurodegenerative conditions. In the present study using anti-1-hexitol-lysi ne (1-HL) antibody, Amadori product, an early glycation product, was detect ed in axonal spheroids in the anterior horn of amyotrophic lateral sclerosi s and in atrophic neurons of spinobulbar muscular atrophy (SBMA, Kennedy di sease with abnormally expanded triplet repeats in androgen receptor gene) b ut not in other regions of the central nervous system. Furthermore, Amadori product was undetectable in the tissues from age-matched controls. Thus, 1 -HL formation could not reflect physiological aging.