Circulating macrophage colony-stimulating factor is not reduced in malignant osteopetrosis.

Abstract:

:Malignant osteopetrosis is a disorder characterized by a deficiency in osteoclast number or function. In one animal model of osteopetrosis, the op/op mouse, macrophage colony-stimulating factor (M-CSF) is absent, and the administration of M-CSF corrects the defects. We evaluated the serum of 13 patients with malignant osteopetrosis by an M-CSF radioimmunoassay to determine if a quantitative M-CSF deficiency existed in these patients. All patients had M-CSF present in levels equal to or higher than control serum. In addition, serum from 6 osteopetrotic patients was tested in a bioassay to determine if the M-CSF present is biologically active, and in all cases there was demonstrable activity in these samples. We provide evidence that deficiency of circulating M-CSF is unlikely to be a major contributor to the etiologic basis for the majority of children with malignant osteopetrosis.

journal_name

Exp Hematol

journal_title

Experimental hematology

authors

Orchard PJ,Dahl N,Aukerman SL,Blazar BR,Key LL Jr

keywords:

subject

Has Abstract

pub_date

1992-01-01 00:00:00

pages

103-5

issue

1

eissn

0301-472X

issn

1873-2399

journal_volume

20

pub_type

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