Gamma Heavy Chain Disease in Man: Translation and Partial Purification of MRNA Coding for the Deleted Protein

Overview

Journal Proc Natl Acad Sci U S A

Specialty Science

Date 1978 Oct 1

PMID 105356

Citations 3

Authors

A Alexander

D Barritault

J Buxbaum

Affiliations

Soon will be listed here.

Abstract

Lymphoid cells obtained from the peripheral blood of a patient with heavy chain disease have been established in long-term culture. They continue to produce a protein antigenically identical to the deleted gamma3 heavy chain disease protein found in the patient's serum. The availability of the cell line has made it possible to analyze the mRNA coding for this protein. The primary in vitro translation product is 1500-2000 daltons larger than the polypeptide portion of the cytoplasmic or secreted protein and has methionine at the amino terminus. The mRNA sediments at 15.5 S on sucrose gradients and therefore appears to be smaller than the 17S message coding for normal-sized mouse gamma chains. It contains a base sequence that codes for a hydrophobic amino-terminal peptide not found in the cytoplasmic protein. There was no evidence for the synthesis of translatable light chain message by these cells. The present data suggest that this protein results from a primary somatic genetic event that gave rise to a cell product bearing a normal aminoterminus sensitive to limited proteolytic digestion. The serum protein thus appears to begin in the hinge region but, in fact, contains a normal heavy chain initiation site.

Citing Articles

gamma Heavy chain disease in man: cDNA sequence supports partial gene deletion model.

Alexander A, Steinmetz M, Barritault D, Frangione B, FRANKLIN E, Hood L Proc Natl Acad Sci U S A. 1982; 79(10):3260-4.

PMID: 6808505 PMC: 346395. DOI: 10.1073/pnas.79.10.3260.

Gamma heavy chain disease in man. Genomic sequence reveals two noncontiguous deletions in a single gene.

Alexander A, Anicito I, Buxbaum J J Clin Invest. 1988; 82(4):1244-52.

PMID: 3139711 PMC: 442675. DOI: 10.1172/JCI113722.

Celluar immunoglobulins in human gamma- and alpha-heavy chain diseases.

PreudHomme J, Brouet J, SELIGMANN M Clin Exp Immunol. 1979; 37(2):283-91.

PMID: 115628 PMC: 1537823.

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