LC1 is a marker for autoimmune hepatitis (AIH) II and is found to be detected in up to 50% of cases. Immunoflourescence of LC1 is often not identified due to the brightness of LKM-1 obscuring accurate visualisation. Some occurrences of LC1 in conjunction with ANA and SMA autoantibodies, in patients with chronic hepatitis C and autoimmune hepatitis, is known. In rare case studies have shown that anti-LC1 is found as the only serological marker in 10% of patients with AIH.Liver Cytosol 1 datasheet
The antigen recognised by LC1 antibodies has been identified as formiminotransferase cyclodeaminase (FTCD). FTCD is a bifunctional enzyme which consists of 8 identical molecular subunits of ~60kDa each arranged in a circular tetramer of dimers. FTCD catalyzes two independent but sequential reactions in the histidine degradation pathway in the mammalian liver. Each subunit consists of an N-terminal transferase active domain and a C-terminal deaminase active domain. The dimers are responsible for the separate activities of both the transferase and deaminase activity.
LC1 reactivity is mainly directed to conformation epitopes with multiple triggers along regions of FTCD, with the majority of activity against distinct epitopes in the N-terminal FT domain. The presence of anti-LC1 antibodies seems to correlate to disease activity unlike LKM-1 in AIH II.
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