Defective lymphocyte glycosidases in the macrophage activation cascade of juvenile osteopetrosis

Nobuto Yamamoto, Venkateswara R. Naraparaju, Paul J. Orchard

Research output: Contribution to journalArticlepeer-review

20 Scopus citations

Abstract

Generation of macrophage-activating factor requires a precursor protein, Gc protein (serum vitamin D3-binding protein), as well as participation of β-galactosidase of inflammation-primed B lymphocytes and sialidase of T lymphocytes. The treatment of human peripheral blood mononuclear cells with an inflammatory lysophospholipid induced β-galactosidase and sialidase activity of lymphocytes, leading to the generation of macrophage-activating factor and activation of monocytes/macrophages. However, lysophospholipid treatment of peripheral blood mononuclear cells from three infantile patients with osteopetrosis resulted in no significant activation of monocytes/macrophages. The lysophospholipid-inducible β-galactosidase activity of B lymphocytes as well as that of the sialidase of T lymphocytes was found to be defective in these patients.

Original languageEnglish (US)
Pages (from-to)1473-1478
Number of pages6
JournalBlood
Volume88
Issue number4
DOIs
StatePublished - Aug 15 1996

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