Biotin-responsive immunoregulatory dysfunction in multiple carboxylase deficiency.
The immunoregulatory system has recently been shown to require prostaglandins (PG) for its activation in man. We report here an impairment of immunoregulatory function, due to defective PGE monocytic production, in a 12-month-old boy with multiple carboxylase deficiency (MCD). The abnormal immune-response was corrected in vitro by adding PGE to the medium. Moreover, PGE deficiency and immunoregulatory dysfunction responded to biotin administration in vivo. It is suggested that the PGE deficiency in MCD could result from an impaired activity of a biotin enzyme, acetyl CoA carboxylase, since the product of this enzyme reaction, malonyl CoA, is required for prostaglandin synthesis.
D001710:Biotin / Q000627:therapeutic use*; D002262:Carboxy-Lyases / Q000172:deficiency*; D003208:Concanavalin A / Q000494:pharmacology; D015232:Dinoprostone; D006801:Humans; D007111:Immunity, Cellular; D007153:Immunologic Deficiency Syndromes / Q000188:drug therapy / Q000201:enzymology* / Q000276:immunology; D007223:Infant; D008213:Lymphocyte Activation; D008297:Male; D009000:Monocytes / Q000276:immunology / Q000378:metabolism; D011458:Prostaglandins E / Q000096:biosynthesis; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
D011458:Prostaglandins E; D003208:Concanavalin A; D001710:Biotin; D002262:Carboxy-Lyases; D015232:Dinoprostone