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Hepatocyte nuclear factor 4 alpha (HNF4 alpha) is an orphan nuclear receptor. HNF4a is expressed in the liver, kidney, intestine, and pancreas. Mutation of HNF4a in humans has been associated with maturity-onset diabetes of the young type 1 (MODY1). HNF4 binds to DNA as an exclusive homodimer. The HNF4a gene is alternatively spliced and may generate up to nine different isoforms, HNF4a1 through HNF4a9.
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