HNF1A (Hepatocyte Nuclear Factor 1 Alpha) is a transcription factor encoded by the *HNF1A* gene, primarily expressed in the liver, pancreas, kidneys, and gastrointestinal tract. It plays a critical role in regulating tissue-specific gene expression, particularly genes involved in glucose metabolism, insulin secretion, and pancreatic β-cell function. Mutations in *HNF1A* are linked to maturity-onset diabetes of the young type 3 (MODY3), a monogenic form of diabetes characterized by autosomal dominant inheritance and early-onset hyperglycemia.
Antibodies targeting HNF1A are essential tools in research and diagnostics. In research, they are used to study HNF1A's expression patterns, localization, and interactions with DNA or other proteins, aiding in understanding its role in development and disease. Clinically, HNF1A antibodies may assist in identifying autoimmune responses in rare cases of autoimmune diabetes or evaluating tissue-specific HNF1A dysfunction. However, unlike diabetes-associated autoantibodies (e.g., GAD65 or IA-2), HNF1A antibodies are not routinely used for diabetes classification. Instead, genetic testing remains the gold standard for confirming *HNF1A*-related MODY. Recent studies also explore HNF1A’s involvement in cancer and renal diseases, expanding the scope of its antibody applications. Challenges include ensuring antibody specificity due to structural similarities with HNF1B. Overall, HNF1A antibodies remain pivotal in unraveling molecular pathways and advancing precision medicine for metabolic disorders.