Discrimination between M2 and M4 antimitochondrial antibodies in primary biliary cirrhosis

10 sera were studied from patients with primary biliary cirrhosis (PBC), that were anomalous in their reactivity against mitochondrial antigens as detected by Western blotting. They had low reactivity against the major, M2 reactive antigen (Mr for beef heart mitochondria, 74 Kd) but reacted against an antigen of Mr 52 Kd (species independent) which was apparently inaccessible in submitochondrial particles (SMP) on ELISA and which was not present in chloroform-released ATPase preparations. In all respects this differed from the characteristics of the M2 antigens and it is concluded that these sera are detecting predominantly the M4-reactive antigen.