SGO1

This protein prevents premature dissociation of the cohesin complex from centromeres during mitosis, ensuring proper chromosome segregation by protecting centromeric cohesin from cleavage until anaphase. Mutations cause Chronic Atrial and Intestinal Dysrhythmia syndrome (characterized by sick sinus syndrome and chronic intestinal pseudo-obstruction typically occurring within the first four decades of life) and Houge-Janssens syndrome 3, with both autosomal dominant and autosomal recessive inheritance patterns reported. The gene is extremely intolerant to loss-of-function variants, reflecting its essential role in cell division.