TY - JOUR
T1 - Defective lymphocyte glycosidases in the macrophage activation cascade of juvenile osteopetrosis
AU - Yamamoto, Nobuto
AU - Naraparaju, Venkateswara R.
AU - Orchard, Paul J.
PY - 1996/8/15
Y1 - 1996/8/15
N2 - 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.
AB - 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.
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U2 - 10.1182/blood.v88.4.1473.bloodjournal8841473
DO - 10.1182/blood.v88.4.1473.bloodjournal8841473
M3 - Article
C2 - 8695868
AN - SCOPUS:0029794020
SN - 0006-4971
VL - 88
SP - 1473
EP - 1478
JO - Blood
JF - Blood
IS - 4
ER -