... metabolism in two lines of thymidinekinase 2-mutated fibroblastsMiriam Frangini1, Chiara Rampazzo1, Elisa Franzolin1, Mari-Carmen Lara2, Maya R. Vila`2,Ramon Martı´2and Vera Bianchi11 ... mito-chondrial inner membrane maintains the cytosolic andmitochondrial compartments in equilibrium.We were interested in investigating whether in the fibroblasts of the two TK2 patients the operation of the ... myopathy. NatGenet 29, 342–344.3 Mandel H, Szargel R, Labay V, Elpeleg O, Saada A, Shalata A, Anbinder Y, Berkowitz D, Hartman C,Barak M et al. (2001) The deoxyguanosine kinase geneis mutated...