Transcriptional corepressor. May specifically inhibit gene expression when recruited to promoter regions by sequence specific DNA-binding proteins such as BCL6 and MLLT3. This repression may be mediated at least in part by histone deacetylase activities which can associate with this corepressor. Involved in the repression of TFAP2A; impairs binding of BCL6 and KDM2B to TFAP2A promoter regions. Via repression of TFAP2A acts as a negative regulator of osteo-dentiogenic capacity in adult stem cells; the function implies inhibition of methylation on histone H3 'Lys-4' (H3K4me3) and 'Lys-36' (H3K36me2). Defects in BCOR are the cause of microphthalmia syndromic type 2 (MCOPS2). Microphthalmia is a clinically heterogeneous disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues (anophthalmia). In many cases, microphthalmia/anophthalmia occurs in association with syndromes that include non-ocular abnormalities. MCOPS2 is a very rare multiple congenital anomaly syndrome characterized by eye anomalies (congenital cataract, microphthalmia, or secondary glaucoma), facial abnormalities (long narrow face, high nasal bridge, pointed nose with cartilages separated at the tip, cleft palate, or submucous cleft palate), cardiac anomalies (atrial septal defect, ventricular septal defect, or floppy mitral valve) and dental abnormalities (canine radiculomegaly, delayed dentition, oligodontia, persistent primary teeth, or variable root length). Belongs to the BCOR family. 4 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Transcription, coactivator/corepressor
Molecular Function: heat shock protein binding; histone deacetylase binding; protein binding; transcription corepressor activity; transcription factor binding; transcription regulatory region DNA binding; ubiquitin-protein transferase activity
Biological Process: chromatin organization; heart development; negative regulation of bone mineralization; negative regulation of histone H3-K36 methylation; negative regulation of histone H3-K4 methylation; negative regulation of transcription from RNA polymerase II promoter; negative regulation of transcription, DNA-dependent; odontogenesis; palate development; regulation of transcription, DNA-templated; specification of axis polarity; transcription, DNA-dependent