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
S. Kikuchi et al., 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, ACT NEUROP, 99(1), 2000, pp. 63-66
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.