Primary structure of human gamma 3 immunoglobulin deletion mutant: gamma 3 heavy-chain disease protein Wis.
The complete sequence of a gamma 3 heavy-chain disease (HCD) protein Wis is presented. The molecule is a dimer of a 289-residue chain linked by 12 disulfide bonds. Protein Wis has an unusual amino terminus, followed by a deletion of most of the VH domain. After a small stretch homologous to the VC joining region, there is a second deletion which ends at the beginning of the quadruplicated hinge. Two carbohydrate groups are linked to Asn-6 and -140. the molecule has an extra interchain disulfide bridge at position 7 in addition to the 11 normally present in the quadruplicated hinge. The previously noted homology to the gamma 1 heavy chain is striking; from positions 224 to 234, protein Wis resembles gamma 1 Nei [Ponstingl, H., & Hilschmann, N. (1976) Hoppe-Seyler's Z. Physiol. Chem. 357, 1571--1604] except for a serine which replaces Asn at position 227. The results, taken together with studies of other immunoglobulin heavy-chain deletion mutants, support the suggestion that the different domains and interdomain regions of human H chains are coded for by different gene segments and that the deleted proteins reflect alterations in the recombination of different genes and/or the splicing of heterogeneous nuclear messenger ribonucleic acid (hn mRNA).
D000595:Amino Acid Sequence; D002872:Chromosome Deletion; D003488:Cyanogen Bromide; D006362:Heavy Chain Disease / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007143:Immunoglobulin Heavy Chains; D007142:Immunoglobulin gamma-Chains; D046911:Macromolecular Substances; D008970:Molecular Weight; D009154:Mutation; D009194:Myeloma Proteins; D010206:Papain; D010434:Pepsin A; D010446:Peptide Fragments / Q000032:analysis
D007143:Immunoglobulin Heavy Chains; D007142:Immunoglobulin gamma-Chains; D046911:Macromolecular Substances; D009194:Myeloma Proteins; D010446:Peptide Fragments; C483997:Wis heavy-chain disease protein, human; C032598:heavy chain disease proteins, human; D010206:Papain; D010434:Pepsin A; D003488:Cyanogen Bromide