Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: Dihydrolipoamide acetyltransferase

Journal Article · Sat Oct 01 00:00:00 EDT 1988 · Proceedings of the National Academy of Sciences of the United States of America; (USA)

DOI:https://doi.org/10.1073/pnas.85.19.7317· OSTI ID:5607044

Coppel, R L; McNeilage, L J; Surh, C D; Van De Water, J; Spithill, T W; Whittingham, S; Gershwin, M E ^[1]

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1. Walter and Eliza Hall Institute of Medical Research, Parkville (Australia)

Primary biliary cirrhosis is a chronic, destructive autoimmune liver disease of humans. Patient sera are characterized by a high frequency of autoantibodies to a M{sub r} 70,000 mitochondrial antigen a component of the M2 antigen complex. The authors have identified a human cDNA clone encoding the complete amino acid sequence of this autoantigen. The predicted structure has significant similarity with the dihydrolipoamide acetyltransferase of the Escherichia coli pyruvate dehydrogenase multienzyme complex. The human sequence preserves the Glu-Thr-Asp-Lys-Ala motif of the lipoyl-binding site and has two potential binding sites. Expressed fragments of the cDNA react strongly with sera from patients with primary biliary cirrhosis but not with sera from patients with autoimmune chronic active hepatitis or sera from healthy subjects.

• OSTI ID: 5607044
• Journal Information: Proceedings of the National Academy of Sciences of the United States of America; (USA), Vol. 85:19; ISSN 0027-8424
• Country of Publication: United States
• Language: English

Related Subjects

59 BASIC BIOLOGICAL SCIENCES
ANTIGENS
MOLECULAR STRUCTURE
LIVER CIRRHOSIS
PATHOGENESIS
TRANSFERASES
AMINO ACID SEQUENCE
LIVER
MAN
MITOCHONDRIA
PHOSPHORUS 32
RATS
RECOMBINANT DNA
ANIMALS
BETA DECAY RADIOISOTOPES
BETA-MINUS DECAY RADIOISOTOPES
BODY
CELL CONSTITUENTS
DAYS LIVING RADIOISOTOPES
DIGESTIVE SYSTEM
DIGESTIVE SYSTEM DISEASES
DISEASES
DNA
ENZYMES
GLANDS
ISOTOPES
LIGHT NUCLEI
MAMMALS
NUCLEI
NUCLEIC ACIDS
ODD-ODD NUCLEI
ORGANIC COMPOUNDS
ORGANOIDS
ORGANS
PHOSPHORUS ISOTOPES
PRIMATES
RADIOISOTOPES
RODENTS
VERTEBRATES
550201* - Biochemistry- Tracer Techniques