LBXCOR1 is a 965 amino acid protein belonging to the SKI family. Localizing to nucleus, LBXCOR1 is highly expressed in the central nervous system (CNS) as well as developing spinal cord and adult brain and testis. LBXCOR1 contains a CH1 domain which is required for transcriptional repression and also acts as a transcriptional co-repressor of LBX1. LBXCOR1 additionally interacts with SMAD1, SMAD2 and SMAD3, and inhibits BMP signaling. Existing as three alternatively spliced isoforms, the gene encoding LBXCOR1 maps to human chromosome 15q23. Encoding more than 700 genes, chromosome 15 is made up of approximately 106 million base pairs and consists of about 3% of the human genome. Angelman and Prader-Willi syndromes, as well as Tay-Sachs disease and Marfan syndorme, are all associated with mutations to chromosome 15.