Family with sequence similarity 193 member A (FAM193A)

Family with sequence similarity 193 member A is a protein encoded by the FAM193A gene located on human chromosome 4p16.3. FAM193A acts as a positive regulator of the p53 tumor suppressor pathway largely by modulating the stability of MDM4, a key negative regulator of p53. Loss of FAM193A leads to increased MDM4 protein levels, suppression of the p53 transcriptional program, and altered cell fate after p53 pathway activation. FAM193A forms a complex with MDM2 and MDM4 in the nucleus and cytoplasm and is essential for full responsiveness to drugs targeting the p53-MDM2/4 axis, such as Nutlin. High FAM193A expression correlates with better prognosis in multiple cancers and with sensitivity to p53-activating agents. Although broadly expressed, FAM193A has also been linked to roles in neurological disease and bone development, but its full physiological functions remain incompletely understood.

Other names

FAM193AC4orf8RES4-22chromosome 4 open reading frame 8protein IT143hypothetical protein LOC86032

Positive regulation of the p53 transcriptional network by destabilizing MDM4, thereby enhancing p53 activity; modulation of response to MDM2/4 inhibitors