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lztr1
LZTR1
LZTR1 is a Cullin3 E3 ubiquitin ligase adaptor that targets RAS GTPases (KRAS, MRAS, RRAS2) for proteasomal degradation; LOF → RAS accumulation → RAS-MAPK hyperactivation; germline biallelic LZTR1 LOF = schwannomatosis; LZTR1 also causes Noonan syndrome (dominant).
Entry Metadata
| Field | Value |
|---|---|
| ID | lztr1 |
| Name | LZTR1 |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Piotrowski A, Xie J, Liu YF, et al. Germline loss-of-function mutations in LZTR1 predispose to an inherited disorder of multiple schwannomas. Nat Genet. 2014;46(2):182-187. · PubMed 24362817
- Steklov M, Pandolfi S, Baietti MF, et al. Mutations in LZTR1 drive human disease by dysregulating RAS ubiquitination. Science. 2018;362(6419):1177-1182. · PubMed 30442762