Genetic defect in biosynthesis of the precursor form of the fourth component of complement.
Under cell-free conditions, liver polysomes from guinea pigs genetically deficient in the fourth component of complement (C4) did not synthesize pro-C4 (the precursor of C4), but did synthesize nascent C4 polypeptides which remained polysome bound. The defect was specific for pro-C4 synthesis since the amounts of total protein and albumin synthesis and release from C4-deficient polysomes were similar to that in normal guinea pig liver polysomes.
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Ina Sue Perlmutter Cystic Fibrosis Center, Children's Hospital Medical Center, Harvard Medical School, Boston, MA 02115, USA.
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D000818:Animals; D002474:Cell-Free System; D003181:Complement C4 / Q000096:biosynthesis* / Q000172:deficiency / Q000235:genetics*; D006168:Guinea Pigs; D047468:Immunoprecipitation; D008099:Liver / Q000276:immunology / Q000378:metabolism*; D011132:Polyribosomes / Q000276:immunology / Q000378:metabolism*; D014176:Protein Biosynthesis; D012333:RNA, Messenger / Q000235:genetics* / Q000378:metabolism
D003181:Complement C4; D012333:RNA, Messenger