LZTR1 facilitates polyubiquitination and degradation of RAS-GTPases

New Noonan syndrome model mice with RIT1 mutation exhibit cardiac hypertrophy and susceptibility to β-adrenergic stimulation-induced cardiac fibrosis

(2019) Shingo Takahara et al.   EBioMedicine

RIT1 oncoproteins escape LZTR1-mediated proteolysis

(2019) Pau Castel et al.   SCIENCE

Novel polyubiquitin imaging system, PolyUb-FC, reveals that K33-linked polyubiquitin is recruited by SQSTM1/p62

(2018) Yoichi Nibe et al.   Autophagy

Autosomal recessive Noonan syndrome associated with biallelic LZTR1 variants

(2018) Jennifer J Johnston et al.   GENETICS IN MEDICINE

Dimerization quality control ensures neuronal development and survival

(2018) Elijah L. Mena et al.   SCIENCE

β‐Catenin‐RAS interaction serves as a molecular switch for RAS degradation via GSK3β

(2018) Sang‐Kyu Lee et al.   EMBO REPORTS

Dominant Noonan syndrome-causing LZTR1 mutations specifically affect the kelch domain substrate-recognition surface and enhance RAS-MAPK signaling

(2018) Marialetizia Motta et al.   HUMAN MOLECULAR GENETICS

Mutations in LZTR1 drive human disease by dysregulating RAS ubiquitination

(2018) M. Steklov et al.   SCIENCE

LZTR1 is a regulator of RAS ubiquitination and signaling

(2018) Johannes W. Bigenzahn et al.   SCIENCE

A novel rasopathy caused by recurrent de novo missense mutations inPPP1CBclosely resembles Noonan syndrome with loose anagen hair

(2016) Karen W. Gripp et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Drugging the undruggables: exploring the ubiquitin system for drug development

(2016) Xiaodong Huang et al.   CELL RESEARCH

Molecular portrait of a rare case of metastatic glioblastoma: somatic and germline mutations using whole-exome sequencing

(2016) Sara Franceschi et al.   NEURO-ONCOLOGY

The BioPlex Network: A Systematic Exploration of the Human Interactome

(2015) Edward L. Huttlin et al.   CELL

Recent advances in RASopathies

(2015) Yoko Aoki et al.   JOURNAL OF HUMAN GENETICS

Rare variants inSOS2andLZTR1are associated with Noonan syndrome

(2015) Guilherme Lopes Yamamoto et al.   JOURNAL OF MEDICAL GENETICS

Heterozygous germline mutations in A2ML1 are associated with a disorder clinically related to Noonan syndrome

(2014) Lisenka ELM Vissers et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

A CULLINary ride across the secretory pathway: more than just secretion

(2014) Albert Lu et al.   TRENDS IN CELL BIOLOGY

Modulation of apoptosis sensitivity through the interplay with autophagic and proteasomal degradation pathways

(2014) M E Delgado et al.   Cell Death & Disease

Gain-of-Function Mutations in RIT1 Cause Noonan Syndrome, a RAS/MAPK Pathway Syndrome

(2013) Yoko Aoki et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Differences in the Regulation of K-Ras and H-Ras Isoforms by Monoubiquitination

(2013) Rachael Baker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The integrated landscape of driver genomic alterations in glioblastoma

(2013) Veronique Frattini et al.   NATURE GENETICS

Germline loss-of-function mutations in LZTR1 predispose to an inherited disorder of multiple schwannomas

(2013) Arkadiusz Piotrowski et al.   NATURE GENETICS

The Ubiquitin Code

(2012) David Komander et al.   Annual Review of Biochemistry

Ubiquitination and selective autophagy

(2012) S Shaid et al.   CELL DEATH AND DIFFERENTIATION

Site-specific monoubiquitination activates Ras by impeding GTPase-activating protein function

(2012) Rachael Baker et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Ras Stabilization Through Aberrant Activation of Wnt/ -Catenin Signaling Promotes Intestinal Tumorigenesis

(2012) W.-J. Jeong et al.   Science Signaling

Ubiquitination of K-Ras Enhances Activation and Facilitates Binding to Select Downstream Effectors

(2011) A. T. Sasaki et al.   Science Signaling

Noonan Syndrome: Clinical Features, Diagnosis, and Management Guidelines

(2010) A. A. Romano et al.   PEDIATRICS

The RASopathies: developmental syndromes of Ras/MAPK pathway dysregulation

(2009) William E Tidyman et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Structures of SPOP-Substrate Complexes: Insights into Molecular Architectures of BTB-Cul3 Ubiquitin Ligases

(2009) Min Zhuang et al.   MOLECULAR CELL