A hereditary immunoglobulin A abnormality: absence of light-heavy—chain assembly: <i>Study of immunoglobulin synthesis in tonsillar cells</i>

Chaya Moroz; Jacob Amir; Andre De Vries

doi:10.1172/JCI106773

Rogoff-Wellcome Medical Research Institute and the Department of Medicine D, Tel-Aviv University Medical School, Beilinson Hospital, Petah Tikva, Israel

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Rogoff-Wellcome Medical Research Institute and the Department of Medicine D, Tel-Aviv University Medical School, Beilinson Hospital, Petah Tikva, Israel

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Rogoff-Wellcome Medical Research Institute and the Department of Medicine D, Tel-Aviv University Medical School, Beilinson Hospital, Petah Tikva, Israel

Find articles by Vries, A. in: JCI | PubMed | Google Scholar

Published in Volume 50, Issue 12 on December 1, 1971
J Clin Invest. 1971;50(12):2726–2733. https://doi.org/10.1172/JCI106773.
© 1971 The American Society for Clinical Investigation

Published December 1, 1971 - Version history

A new immunoglobulin A abnormality, absence of assembly of α-chain and light-chain, was found in an adult female suffering from recurrent upper respiratory infection and tonsillitis since childhood, but otherwise healthy. The IgA abnormality was manifest in her serum by the presence of free α-chains, in her saliva by the presence of α-chains bound to secretory piece, and in her urine by the presence of free α-chains and free light-chains. The serum IgG and IgM were found to be complete, containing both heavy-chains and light-chains.

Evidence for this immunoglobulin A abnormality was also found in the proposita's mother and elder son, demonstrating it to be a hereditary disorder.

Studies performed with patient's tonsillar cells in short-term culture, using amino acids-¹⁴C, revealed synthesis and secretion of both free α-chains and free light-chains, in addition to synthesis and secretion of normally assembled IgG and IgM.

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