Cloning and characterization of the cDNA coding for a polymyositis-scleroderma overlap syndrome-related nucleolar 100-kD protein.
About 50% of patients with the polymyositis-scleroderma overlap syndrome are reported to have autoantibodies to a nucleolar particle termed PM/Scl. The particle consists of several polypeptides of which two proteins of 75 and 100 kD have been identified as the major antigenic components. Here we report on the cDNA cloning and partial epitope mapping of the 100-kD autoantigen from human placenta and HeLa lambda gt11 libraries. The deduced amino acid sequence encodes a protein of 885 amino acid residues with a molecular mass of 100.8 kD. Rabbit antibodies raised against a recombinant protein fragment reacted in immunofluorescence and immunoblotting in the same manner as human autoantibodies directed against the nucleolar 100-kD protein. Sequence analysis shows close homology to a consensus sequence of 12 amino acids from serine/threonine kinases, suggesting a possible function for this autoantigen. A major antigenic region is found to be located within the NH2-terminal third of the polypeptide.
Blüthner
M
M
Institute of Molecular Genetics, University of Heidelberg, Germany.
Bautz
F A
FA
D000595:Amino Acid Sequence; D000818:Animals; D001324:Autoantigens / Q000096:biosynthesis / Q000097:blood / Q000235:genetics*; D001483:Base Sequence; D002466:Cell Nucleolus / Q000502:physiology*; D003001:Cloning, Molecular; D003240:Connective Tissue Diseases / Q000235:genetics*; D004247:DNA / Q000235:genetics / Q000302:isolation & purification; D000939:Epitopes / Q000032:analysis; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; D015723:Gene Library; D006367:HeLa Cells; D006801:Humans; D015151:Immunoblotting; D008969:Molecular Sequence Data; D009687:Nuclear Proteins / Q000096:biosynthesis / Q000097:blood / Q000235:genetics*; D010920:Placenta / Q000378:metabolism; D016133:Polymerase Chain Reaction / Q000379:methods; D017285:Polymyositis / Q000235:genetics*; D011994:Recombinant Proteins / Q000096:biosynthesis / Q000302:isolation & purification; D015183:Restriction Mapping; D017386:Sequence Homology, Amino Acid; D012871:Skin Diseases / Q000235:genetics*; D013577:Syndrome
D001324:Autoantigens; D000939:Epitopes; D009687:Nuclear Proteins; D011994:Recombinant Proteins; D004247:DNA; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human