T cell physiopathology

DESCRIPTION OF THE OFFER: Blood cells, especially erythrocytes, are protected from complement deposition and lysis by membrane regulators such as CD46/MCP. Somatic or congenital loss-of-function mutations in CD46 may cause atypical haemolytic uremic syndrome (aHUS). We believe aHUS patient-derived B cell lines could be useful as screening biosensors for the lytic activity of plasma samples with dysfunctional regulation of complement activation, for instance, due to factor H deficiency.

Aims and activities:

1)      To generate Epstein-Barr virus (EBV)-immortalized B cell lines from aHUS patients with CD46/MCP deficiency.

2)      To analyse C3/C3b deposition by flow cytometry on these EBV cell lines after incubation with factor H-deficient plasma.

3)      To validate and eventually commercialize these cell lines as biosensors of plasma C3 dysregulation in plasma samples from patients with complement disorders.