Serologically defined V region subgroups of human lambda light chains.
The availability of numerous antisera prepared against lambda-type Bence Jones proteins and lambda chains of known amino acid sequence has led to the differentiation and classification of human lambda light chains into one of five V lambda subgroups. The five serologically defined subgroups, V lambda I, V lambda II, V lambda III, V lambda IV, and V lambda VI, correspond to the chemical classification that is based on sequence homologies in the first framework region (FR1). Proteins designated by sequence as lambda V react with specific anti-lambda II antisera and are thus included in the V lambda II subgroup classification. The isotypic nature of the five V lambda subgroups was evidenced through analyses of lambda-type light chains that were isolated from the IgG of normal individuals. Based on analyses of 116 Bence Jones proteins, the frequency of distribution of the lambda I, lambda II/V, lambda III, lambda IV, and lambda VI proteins in the normal lambda chain population is estimated to be 27%, 37%, 23%, 3%, and 10%, respectively. This distribution of V lambda subgroups was comparable to that found among 82 monoclonal Ig lambda proteins. Considerable V lambda intragroup antigenic heterogeneity was also apparent. At least two sub-subgroups were identified among each of the five major V lambda subgroups, implying the existence of multiple genes in the human V lambda genome. The V lambda classification of 54 Ig lambda proteins obtained from patients with primary or multiple myeloma-associated amyloidosis substantiated the preferential association of lambda VI light chains with amyloidosis AL and the predominance of the normally rare V lambda VI subgroup in this disease.
D000686:Amyloidosis / Q000209:etiology / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000235:genetics / Q000276:immunology; D001536:Bence Jones Protein / Q000235:genetics / Q000276:immunology; D006801:Humans; D007106:Immune Sera; D005779:Immunodiffusion; D007127:Immunoglobulin Constant Regions / Q000235:genetics / Q000276:immunology; D007074:Immunoglobulin G / Q000235:genetics / Q000276:immunology; D007135:Immunoglobulin Variable Region / Q000145:classification* / Q000235:genetics / Q000276:immunology; D007146:Immunoglobulin lambda-Chains / Q000145:classification* / Q000235:genetics / Q000276:immunology; D009101:Multiple Myeloma / Q000150:complications / Q000276:immunology; D009194:Myeloma Proteins / Q000235:genetics / Q000276:immunology
D000911:Antibodies, Monoclonal; D007106:Immune Sera; D007127:Immunoglobulin Constant Regions; D007074:Immunoglobulin G; D007135:Immunoglobulin Variable Region; D007146:Immunoglobulin lambda-Chains; D009194:Myeloma Proteins; D001536:Bence Jones Protein