Ataxin-1-like (ATXN1L)

Ataxin-1-like (ATXN1L) is a chromatin-binding transcriptional corepressor protein and a paralog of Ataxin-1 (ATXN1). It enables chromatin binding and RNA binding activities and participates in the negative regulation of transcription mediated by RNA polymerase II. ATXN1L cooperates with Capicua (CIC) and ATXN1 in multimeric nuclear complexes, notably repressing Notch signaling by acting as a corepressor with CBF1. This protein plays regulatory roles in learning, memory, and social behavior, and is involved in development of the nervous system[2][3]. Unlike ATXN1, it does not encode a polyglutamine (polyQ) tract, but shares key structural domains with ATXN1 (e.g., AXH domain) important for protein-protein interactions[1][2]. While ATXN1L can suppress ATXN1-mediated cytotoxicity in spinocerebellar ataxia type 1, its primary functions are regulatory and it is not considered a direct therapeutic target[2][3]. The gene is associated with some CNS tumor types, but no approved drugs or biomarkers specifically target or monitor ATXN1L[2].

Other names

Ataxin-1-likeATXN1LBrother of ATXN1BOATBOAT1Brother of ataxin-1

Not applicable; no drugs directly target ATXN1L or act through its modulation