Long-Term culture and T cell receptor analysis of T cell clones isolated from a patient with adenosine deaminase deficiency and type 1 diabetes

We performed limiting dilution culture of T cells from a patient affected by primary immunodeficiency as a result of complete lack of adenosine deaminase (ADA) activity and also affected by insulin-dependent diabetes mellitus (type 1diabetes). despite the occurrence of immunoideficiency, we were able to raise and grow T cell clones derived fromthis patient in long term culture. These T cells displayed ADA enzymatic activity and produced interleukin-2 after engagement of their T cell receptor (TCR)/CD3 complex. we analyzed the TCR repertoire of such clones by nucleotide sequencing of TCR beta chains. The results show that the T cell clones express different Vbeta but similar J regions. However, the CDR3 regions which are implicated in antigen recognition were found heterogeneous.