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ProteomicsLztr1 loss leads to Noonan syndrome-like vascular defects by dysregulating vesicular trafficking
Mutations in LZTR1, an adaptor for cullin 3 (CUL3) ubiquitin ligase complex, are associated with glioma, hepatocarcinoma, paediatric cancers, Schwannomatosis, and Noonan syndrome (NS). NS is a poorly understood and complex disease. The variety of NS phenotypes makes it challenging to elucidate the m...
ORGANISM(S): Homo sapiens (Human) 
2020-10-14 | PXD011926 | Pride
Hela cells Vegf Noonan syndrome Lztr1 Vesicular trafficking Ubiquitin Chmp1b 
ProteomicsLoss-of-function of ubiquitin ligase adaptor LZTR1 triggers human disease by dysregulating RAS ubiquitination
Mutations in LZTR1, a substrate adaptor for cullin 3 (CUL3) ubiquitin ligase complexes, have been recently associated with Noonan syndrome and familial Schwannomatosis. Concordantly, we found that Lztr1+/- mice showed craniofacial abnormalities, cardio defects, and premature ageing; whereas loss of ...
ORGANISM(S): Mus musculus (Mouse) 
2018-11-07 | PXD007035 | Pride
Ras signalling Schwannomatosis Noonan syndrome Ubiquitin 
ProteomicsLZTR1 is a Melanoma Oncogene that Promotes Cell Growth and Inhibits Apoptosis Under Stress
LZTR1 is known to act as a tumor suppressor in glioma, acting as an adaptor protein for Cullin 3 (CUL3) ubiquitin ligase. However, we demonstrated that LZTR1 is amplified in acral melanomas, and is required for melanoma cell proliferation and anchorage-independent growth. To understand the molecular...
ORGANISM(S): Homo sapiens (Human) 
2025-10-13 | PXD054157 | Pride
Autophagy Stress response Lztr1 Acral melanoma 
ProteomicsRIT1 oncoproteins escape LZTR1-mediated proteolysis
These data accompany our publication "RIT1 oncoproteins escape LZTR1-mediated proteolysis" (Castel P., et al. Science, 2019). Experiment 1: Identification of LZTR1 as an interacting partner of RIT1 Q Exactive Plus P20161208-02 FLAG GFP P20161208-03 FLAG RIT1 LTQ Orbitrap Velos ...
ORGANISM(S): Homo Sapiens (ncbitaxon:9606) 
2019-02-14 | MSV000083444 | MassIVE
ProteomicsLoss-of-function of ubiquitin ligase adaptor LZTR1 triggers human disease by dysregulating RAS ubiquitination
Mutations in LZTR1, a substrate adaptor for cullin 3 (CUL3) ubiquitin ligase complexes1, have been recently associated with Noonan syndrome2,3 and familial Schwannomatosis4-6. Concordantly, we found that Lztr1+/- mice showed craniofacial abnormalities, cardio defects, and premature ageing; whereas l...
ORGANISM(S): Homo sapiens (Human) 
2018-11-04 | PXD007049 | Pride
Virotrap protein complex 
GenomicsCellular Crosstalk Drives Hypertrophic Cardiomyopathy in LZTR1 Deficiency
Cellular Crosstalk Drives Hypertrophic Cardiomyopathy in LZTR1 Deficiency
PRJNA1133071 | ENA
GenomicsPhosphorylation Protects Oncogenic RAS from LZTR1-Mediated
Phosphorylation Protects Oncogenic RAS from LZTR1-Mediated
PRJNA1502817 | ENA
GenomicsQuantitative Analysis of Wild Type and Lztr1 +/- Transcriptomes in MEFs (murine embryonic fibroblasts)
Quantitative Analysis of Wild Type and Lztr1 +/- Transcriptomes in MEFs (murine embryonic fibroblasts)
PRJNA507373 | ENA
OtherPhosphorylation Protects Oncogenic RAS from LZTR1-Mediated
Oncogenic KRAS and NRAS mutations are common in hematologic malignancies, but how they signal in that context is less well characterized than in carcinomas. To uncover novel RAS biology and potential therapeutic vulnerabilities, we employed a multi-omics screening approach in multiple myeloma to ide...
ORGANISM(S): Homo sapiens 
2026-08-11 | GSE341488 | GEO
UnknownExpanding the mutational spectrum of LZTR1 in schwannomatosis.
Not available
S-EPMC4463507 | biostudies-literature
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