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RRAS2

Function

GTP-binding protein with GTPase activity involved in the regulation of MAPK signaling pathway, thereby controlling multiple cellular processes (PubMed:31130282). Involved in the regulation of MAPK signaling pathway (PubMed:31130282, PubMed:31130285). Regulation of craniofacial development (PubMed:31130282, PubMed:31130285).

Involvement in disease

Ovarian cancer

OC

The term ovarian cancer defines malignancies originating from ovarian tissue. Although many histologic types of ovarian tumors have been described, epithelial ovarian carcinoma is the most common form. Ovarian cancers are often asymptomatic and the recognized signs and symptoms, even of late-stage disease, are vague. Consequently, most patients are diagnosed with advanced disease.

None

Disease susceptibility is associated with variants affecting the gene represented in this entry.

Noonan syndrome 12

NS12

A form of Noonan syndrome, a disease characterized by short stature, facial dysmorphic features such as hypertelorism, a downward eyeslant and low-set posteriorly rotated ears, and a high incidence of congenital heart defects and hypertrophic cardiomyopathy. Other features can include a short neck with webbing or redundancy of skin, deafness, motor delay, variable intellectual deficits, multiple skeletal defects, cryptorchidism, and bleeding diathesis. Individuals with Noonan syndrome are at risk of juvenile myelomonocytic leukemia, a myeloproliferative disorder characterized by excessive production of myelomonocytic cells. NS12 inheritance is autosomal dominant. There is considerable variability in severity.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

May be post-translationally modified by both palmitoylation and polyisoprenylation.

Fatty-acylation at Lys-192, Lys-194; lys-196 and Lys-197 is required for localization to the plasma membrane and activity (PubMed:28406396). Defatty-acylated by SIRT6, affecting its localization to the plasma membrane (PubMed:28406396).

Sequence Similarities

Belongs to the small GTPase superfamily. Ras family.

Tissue Specificity

Ubiquitously present in all tissues examined, with the highest levels in heart, placenta, and skeletal muscle. Moderate levels in lung and liver; low levels in brain, kidney, and pancreas.

Cellular localization

Alternative names

TC21, RRAS2, Ras-related protein R-Ras2, Ras-like protein TC21, Teratocarcinoma oncogene

swissprot:P62070 omim:600098 entrezGene:22800