Abstract
The ultraviolet (UV) radiation resistance-associated gene (UVRAG) was initially identified for its capability to partially complement UV sensitivity in xeroderma pigmentosum (XP) cells. Subsequently, UVRAG is recognized as a tumor suppressor and autophagy-related protein. In recent years, substantial bodies of work provide new insight into UVRAG structure and multiple biological functions. UVRAG consists of a proline-rich domain, a calcium-dependent phospholipid binding C2 domain, a coiled- coil domain, a DNA-dependent protein kinase binding domain and a CEP-63 binding domain. In addition to its tumor suppression activity, UVRAG regulates autophagy and endocytic trafficking, participates in apoptosis, maintains genomic stability, gets involved in organ rotation during development. In the heart, UVRAG is required to maintain cardiac structure and function. UVRAG deficiency in the heart leads to age- related dilated cardiomyopathy. In this review, we summarize these recent findings of UVRAG that are shedding new light on its crucial roles in many biological processes and implications for the pathophysiology of various diseases including heart disease.
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