Abstract:
:Human leucocytes contain two different MU-NANA neuraminidases, which can be distinguished by Concanavalin A binding. The Con A binding form is predominant in lymphocytes (more than 80%) and the non-binding form predominates in granulocytes. The pH optima of both these neuraminidases as well as their subcellular localization as determined by Percoll gradient centrifugation suggest that they are both lysosomal. Immunological studies indicate that the Con A binding form is present in a complex with beta-galactosidase whereas the non-binding form is not. Leucocytes from patients with sialidosis or galactosialidosis are deficient in the Con A binding neuraminidase, whereas the non-binding form is normal. In sialolipidosis both forms are normal. These results demonstrate that leucocytes contain at least two genetically different MU-NANA neuraminidases. Thus, the use of leucocytes should be avoided for the diagnosis of sialidosis and galactosialidosis, and isolated lymphocytes should be used to obtain reliable results.
journal_name
Biochem Biophys Res Communjournal_title
Biochemical and biophysical research communicationsauthors
Verheijen FW,Janse HC,van Diggelen OP,Bakker HD,Loonen MC,Durand P,Galjaard Hdoi
10.1016/0006-291x(83)91224-xsubject
Has Abstractpub_date
1983-12-16 00:00:00pages
470-8issue
2eissn
0006-291Xissn
1090-2104pii
0006-291X(83)91224-Xjournal_volume
117pub_type
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