Liver Cytosol 1

Product Information

LC-1 is a marker for autoimmune hepatitis (AIH) type II and antibodies against LC-1 are found to be detected in up to 50% of cases. Immunofluorescence of LC-1 is often not identified due to the brightness of LKM-1 obscuring accurate visualisation. Some occurrences of anti-LC-1 in conjunction with antinuclear antibodies (ANA) and smooth muscle antibodies (SMA) in patients with chronic hepatitis C and autoimmune hepatitis, is known. Some case studies have shown that anti-LC-1 is found as the only serological marker in patients with AIH type II.

The antigen recognised by LC-1 antibodies has been identified as formiminotransferase cyclodeaminase (FTCD). FTCD is a bifunctional enzyme which consists of 8 identical molecular subunits of 60 kDa each arranged in a circular tetramer of dimers. FTCD catalyses 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.

Clinical Indications

Autoimmune Hepatitis

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MSDS

References

1. MacKenzie RE, Aldridge M, Paquin J., (1980) The bifunctional enzyme formiminotransferase-cyclodeaminase is a tetramer of dimers. J. Biol. Chem. 255:200-211
2. Muratori L et al., (1995) Detection of anti-liver cytosol antibody type 1 (anti-LC1) by immunodiffusion, counterimmunoelectrophoresis and immunoblotting: comparison of different techniques. J. Immunol. Meth. 187: 259-63
3. Muratori L et al., (1998) Liver/kidney microsomal antibody type 1 and liver cytosol antibody type 1 concentrations in type 2 autoimmune hepatitis. Gut 42:721-6
4. Muratori L et al., (2001) Distinct epitopes on formiminotransferase cyclodeaminase induce autoimmune liver cytosol antibody type 1. Hepatology 34:494-501
5. Beland K et al., (2004) Anti-LC1 autoantibodies in patients with chronic hepatitis C virus infection. J. Autoimmun. 22:159-66
6. Lenzi M et al., (1995) Liver cystosolic 1 antigen-antibody system in type 2 autoimmune hepatitis and hepatitis C virus infection. Gut 36: 749-54
7. Murley LL, Mejia NR, MacKenzie RE (1993) The nucleotide sequence of porcine formiminotransferase cyclodeaminase. Expression and purification from Escherichia coli. J. Biol. Chem. 268: 22820-4
8. Kohls D et al., (2000) The crystal structure of the formiminotransferase domain of formiminotransferase cyclodeaminase: implications for substrate channeling in a bifunctional enzyme. Structure 8: 35-46
9. Lapierre P et al., (1999) Formiminotransferase cyclodeaminase is an organ-specific autoantigen recognized by sera of patients with autoimmune hepatitis. Gastroenterol. 116:643-9
10. Martini E et al., (1988) Antibody to liver cytosol (anti-LC1) in patients with autoimmune chronic hepatitis type 2. Hepatol. 8:1662-6
11. Renous R et al.,(2004) Characterization of the antigenicity of the formiminotransferase-cyclodeaminase in type 2 autoimmune hepatitis. Exp. Cell Res. 292: 332-41
12. Rigopoulou EI et al., (2007) Autoimmune hepatitis-specific antibodies against soluble liver antigen and liver cytosol type 1 in patients with chronic viral hepatitis. J. Autoimmun. Dis. 4:2-7