Hras

HRAS is the acronym for "Harvey-Rat-Sarcoma". The Harvey-Rat-Sarcoma protein belongs to the family of RAS proteins (see RAS below) and was first identified as a transforming oncogene in 1982.

HRas acts as a central switch point in a number of signal transduction pathways involved in the regulation of cell growth and differentiation. This key role in the development of tumours has made the HRas protein an important target in the development of oncological drugs. Mutations in the encoding HRAS gene lead to a pathological gene product.

The sebaceous nevus is caused by postzygotic HRAS mutations in 95% of cases. HRAS mutations are also found in Schimmelpenning syndrome (sebaceous nevus in combination with ophthalmological, CNS disorders, osseous and cardiac malformations) in the organoid nevus of the skin and in the affected organs (Groesser L et al. 2012). Postzygotic HRAS mutations are also found in phacomatosis pigmentokeratotica in both the organoid and melanocytic nevus.

1. Groesser L et al (2012) Postzygotic HRAS and KRAS mutations cause nevus sebaceous and Schimmelpenning syndrome.Nat Genet 44:783-787.
2. Groesser L et al (2013) Phacomatosis pigmentokeratotica is caused by a postzygotic HRAS mutation in a multipotent progenitor cell. J Invest Dermatol 133:1998-2003.