Evidence for the existence of an actin-derived protein in ataxia-telangiectasia lymphoblastoid cell lines.

In a number of lymphoblastoid cell lines from individuals with the genetic disease ataxia-telangiectasia (A-T), a decrease in the levels of actin and a concomitant increase in the levels of a protein species of 37K has been observed to occur following high cell density. In this paper we describe ataxia-telangiectasia lymphoblastoid lines that show this response constitutively, and demonstrate that this 37K protein is most likely an actin-derived peptide. This is because of extensive similarity between the two proteins in the spectrum of peptides produced by proteolytic fingerprinting. It appears likely that this 37K protein is similar to the well documented protease-resistant actin fragments produced by protease digestion of actin in vitro. Examination of a number of possible initiators found no clear signal that promoted this response, although the response is clearly very dependent on cell culture conditions. The possible involvement of a cytoskeletal anomaly in ataxia-telangiectasia is discussed.