Leucine-zipper-like transcriptional regulator 1

Definition:

Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin-protein ligase complex that mediates ubiquitination of Ras (K-Ras/KRAS, N-Ras/NRAS and H-Ras/HRAS). Is a negative regulator of RAS-MAPK signaling that acts by controlling Ras levels and decreasing Ras association with membranes.

References:

[1]. Johannes W Bigenzahn, et al. LZTR1 is a regulator of RAS ubiquitination and signaling. Science. 2018 Dec 7;362(6419):1171-1177. [Content Brief]

[2]. M Steklov, et al. Mutations in LZTR1 drive human disease by dysregulating RAS ubiquitination. Science. 2018 Dec 7;362(6419):1177-1182. [Content Brief]

[3]. Marialetizia Motta, et al. Dominant Noonan syndrome-causing LZTR1 mutations specifically affect the Kelch domain substrate-recognition surface and enhance RAS-MAPK signaling. Hum Mol Genet. 2019 Mar 15;28(6):1007-1022. [Content Brief]

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