Constant region of a kappa III immunoglobulin light chain as a major AL-amyloid protein.
AL-amyloidoses are generally described as a group of disorders in which N-terminal fragments of monoclonal immunoglobulin light chains are transferred into amyloid fibrils. We have, by amino acid sequence analyses and immunological methods, characterized the Bence-Jones protein and the corresponding AL protein as a kappa III immunoglobulin light chain from material of a patient with systemic AL-amyloidosis presenting as a local inguinal tumour. The two proteins showed some unique features. The major part of the AL amyloid fibril protein consisted of C-terminal fragments of the Bence-Jones protein. Furthermore, both the Bence-Jones protein and the AL protein were glycosylated, with possibly a glycosylation in the constant part of the light chain.
Engvig
J P
JP
Department of Biochemistry and Biotechnology Center of Oslo, University of Oslo, Norway.
Olsen
K E
KE
Gislefoss
R E
RE
Sletten
K
K
Wahlström
O
O
Westermark
P
P
D000368:Aged; D000595:Amino Acid Sequence; D000682:Amyloid / Q000737:chemistry* / Q000276:immunology; D001536:Bence Jones Protein / Q000737:chemistry* / Q000276:immunology; D002241:Carbohydrates / Q000032:analysis; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D015151:Immunoblotting; D007127:Immunoglobulin Constant Regions / Q000737:chemistry* / Q000276:immunology; D007145:Immunoglobulin kappa-Chains / Q000737:chemistry* / Q000276:immunology; D008969:Molecular Sequence Data; D017386:Sequence Homology, Amino Acid
D000682:Amyloid; D002241:Carbohydrates; D007127:Immunoglobulin Constant Regions; D007145:Immunoglobulin kappa-Chains; C035222:amyloid protein AL; D001536:Bence Jones Protein