The ATN1 antibody targets atrophin-1. a protein encoded by the *ATN1* gene, which is implicated in transcriptional regulation and neuronal survival. Atrophin-1 is ubiquitously expressed but plays a critical role in nervous system development. It interacts with various nuclear receptors and corepressors, influencing gene expression pathways. Mutations in *ATN1*, particularly CAG trinucleotide repeat expansions, are linked to dentatorubral-pallidoluysian atrophy (DRPLA), a rare autosomal dominant neurodegenerative disorder. DRPLA is characterized by ataxia, myoclonus, epilepsy, and cognitive decline, resembling Huntington's disease.
ATN1 antibodies are primarily used in research to study the protein's localization, expression levels, and pathological mechanisms in DRPLA and related conditions. They enable detection of atrophin-1 in tissues or cell lines via techniques like Western blotting, immunohistochemistry, and immunofluorescence. Studies using these antibodies have revealed aberrant nuclear accumulation of mutant atrophin-1. contributing to neuronal toxicity. Additionally, ATN1 antibodies aid in exploring interactions with binding partners, shedding light on its role in cellular homeostasis. Their application is crucial for advancing therapeutic strategies targeting *ATN1*-associated disorders.