Immunochemical quantification of glycoconjugates in serum and cerebrospinal fluid of amyotrophic lateral sclerosis patients.
Glycoconjugates in the serum of 73 patients with amyotrophic lateral sclerosis (ALS), 21 cases of other motor neuron diseases and 20 healthy controls were determined. Cerebrospinal fluid (CSF) was studied in 64, 7 and 10 of these subjects, respectively. The level of sialic acid containing glycoconjugates, detected by Maakia amurensis agglutinin (MAA), was decreased in the serum of 61.6% of the ALS patients, while in the CSF it was decreased, on average, in 75% of these cases. Only in single ALS cases was the concentration of these glycoconjugates increased. There was no correlation between the content of MAA-labelled glycoconjugates both in serum and CSF and the titre of sialic acid containing anti-GM1 gangliosides. The glycoconjugates, detected by peanut agglutinin (PNA) which recognizes the disaccharide galactose beta(1-3)N- acetylgalactosamine (GGN), were decreased in the serum of 78.1% of ALS patients, while in CSF they were increased in 54.7% of these cases. There was no correlation between the concentration of PNA-labelled glycoconjugates both in serum and CSF as well as the titre of antibodies against GGN-containing anti-GM1 and anti- AGM1 gangliosides. Changes in the level of the MAA- and PNA- labelled glycoconjugates, as well as the titre of anti-GM1 and anti-AGM1 gangliosides antibodies were not specific for ALS. They were also observed in some cases of other motor neuron diseases. The low level of the lectin-labelled glycoconjugates in serum and partly in CSF of the majority of ALS patients is possibly the consequence of their accelerated clearance and/or specific inactivation by the formation of immune complexes or epitope binding. Degeneration of neurons and muscle cells could also be responsible. The relatively low incidence of high anti- glycolipids antibodies titre may be, at least partly, connected with the low concentration of the appropriate antigens. The increased content of PNA-labelled glycoconjugates in the CSF of the majority of ALS patients, together with the low incidence of high titre of antibodies against the appropriate glycolipids, could indicate that in CSF this lectin binds to the GGN epitope of glycoproteins rather than to the GGN epitope of glycolipids.
Niebroj-Dobosz
I
I
Department of Neurology, Medical University of Warsaw and Neuromuscular Unit Medical Research Centre, Polish Academy of Sciences, Warsaw, Poland.
Janik
P
P
Jamrozik
Z
Z
Kwiecinski
H
H
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000276:immunology*; D005260:Female; D006001:Glycoconjugates / Q000097:blood* / Q000134:cerebrospinal fluid*; D006017:Glycolipids / Q000097:blood / Q000134:cerebrospinal fluid; D006023:Glycoproteins / Q000097:blood / Q000134:cerebrospinal fluid; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged
D006001:Glycoconjugates; D006017:Glycolipids; D006023:Glycoproteins