OTULIN allies with LUBAC to govern angiogenesis by editing ALK1 linear polyubiquitin

OTULIN protects the liver against cell death, inflammation, fibrosis, and cancer

(2020) Rune Busk Damgaard et al.   CELL DEATH AND DIFFERENTIATION

Fragment-based covalent ligand screening enables rapid discovery of inhibitors for the RBR E3 ubiquitin ligase HOIP

(2019) Henrik Johansson et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist's perspective

(2018) Athena Kritharis et al.   HAEMATOLOGICA

Hereditary hemorrhagic telangiectasia: diagnosis and management from the hematologist's perspective

(2018) Athena Kritharis et al.   HAEMATOLOGICA

OTULIN limits cell death and inflammation by deubiquitinating LUBAC

(2018) Klaus Heger et al.   NATURE

LUBAC is essential for embryogenesis by preventing cell death and enabling haematopoiesis

(2018) Nieves Peltzer et al.   NATURE

Decreased Expression of Vascular Endothelial Growth Factor Receptor 1 Contributes to the Pathogenesis of Hereditary Hemorrhagic Telangiectasia Type 2

(2018) Jérémy H. Thalgott et al.   CIRCULATION

The Met1-Linked Ubiquitin Machinery: Emerging Themes of (De)regulation

(2017) Matous Hrdinka et al.   MOLECULAR CELL

TGF-β Signaling in Control of Cardiovascular Function

(2017) Marie-José Goumans et al.   Cold Spring Harbor Perspectives in Biology

Regulation of the ALK1 ligands, BMP9 and BMP10

(2016) W. Li et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

The Deubiquitinase OTULIN Is an Essential Negative Regulator of Inflammation and Autoimmunity

(2016) Rune Busk Damgaard et al.   CELL

SPATA2 Links CYLD to LUBAC, Activates CYLD, and Controls LUBAC Signaling

(2016) Paul R. Elliott et al.   MOLECULAR CELL

Biallelic hypomorphic mutations in a linear deubiquitinase define otulipenia, an early-onset autoinflammatory disease

(2016) Qing Zhou et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Signaling Receptors for TGF-β Family Members

(2016) Carl-Henrik Heldin et al.   Cold Spring Harbor Perspectives in Biology

Human HOIP and LUBAC deficiency underlies autoinflammation, immunodeficiency, amylopectinosis, and lymphangiectasia

(2015) Bertrand Boisson et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Functional and splicing defect analysis of 23 ACVRL1 mutations in a cohort of patients affected by Hereditary Hemorrhagic Telangiectasia

(2015) Ferdos Alaa el Din et al.   PLoS One

LUBAC-Recruited CYLD and A20 Regulate Gene Activation and Cell Death by Exerting Opposing Effects on Linear Ubiquitin in Signaling Complexes

(2015) Peter Draber et al.   Cell Reports

Anti-angiogenic therapeutic strategies in hereditary hemorrhagic telangiectasia

(2015) Daniela S. Ardelean et al.   Frontiers in Genetics

Hereditary hemorrhagic telangiectasia: genetics and molecular diagnostics in a new era

(2015) Jamie McDonald et al.   Frontiers in Genetics

Binding of OTULIN to the PUB Domain of HOIP Controls NF-κB Signaling

(2014) Veronique Schaeffer et al.   MOLECULAR CELL

Molecular Basis and Regulation of OTULIN-LUBAC Interaction

(2014) Paul R. Elliott et al.   MOLECULAR CELL

Linear ubiquitin chains: NF-κB signalling, cell death and beyond

(2014) Kazuhiro Iwai et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The role of BMPs in endothelial cell function and dysfunction

(2014) Laura A. Dyer et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

HOIP Deficiency Causes Embryonic Lethality by Aberrant TNFR1-Mediated Endothelial Cell Death

(2014) Nieves Peltzer et al.   Cell Reports

OTU Deubiquitinases Reveal Mechanisms of Linkage Specificity and Enable Ubiquitin Chain Restriction Analysis

(2013) Tycho E.T. Mevissen et al.   CELL

OTULIN Antagonizes LUBAC Signaling by Specifically Hydrolyzing Met1-Linked Polyubiquitin

(2013) Kirstin Keusekotten et al.   CELL

ALK1-Smad1/5 signaling pathway in fibrosis development: Friend or foe?

(2013) José M. Muñoz-Félix et al.   CYTOKINE & GROWTH FACTOR REVIEWS

SnoN facilitates ALK1–Smad1/5 signaling during embryonic angiogenesis

(2013) Qingwei Zhu et al.   JOURNAL OF CELL BIOLOGY

OTULIN Restricts Met1-Linked Ubiquitination to Control Innate Immune Signaling

(2013) Berthe Katrine Fiil et al.   MOLECULAR CELL

The linear ubiquitin-specific deubiquitinase gumby regulates angiogenesis

(2013) Elena Rivkin et al.   NATURE

The Ubiquitin Code

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

Structure of the Alk1 Extracellular Domain and Characterization of Its Bone Morphogenetic Protein (BMP) Binding Properties

(2012) Pardeep Mahlawat et al.   BIOCHEMISTRY

ALK1 Signaling Inhibits Angiogenesis by Cooperating with the Notch Pathway

(2012) Bruno Larrivée et al.   DEVELOPMENTAL CELL

The E3 ligase HOIP specifies linear ubiquitin chain assembly through its RING-IBR-RING domain and the unique LDD extension

(2012) Judith J Smit et al.   EMBO JOURNAL

Regulation of NF-κB by deubiquitinases

(2012) Edward W. Harhaj et al.   IMMUNOLOGICAL REVIEWS

Specificity and Structure of a High Affinity Activin Receptor-like Kinase 1 (ALK1) Signaling Complex

(2012) Sharon A. Townson et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Immunodeficiency, autoinflammation and amylopectinosis in humans with inherited HOIL-1 and LUBAC deficiency

(2012) Bertrand Boisson et al.   NATURE IMMUNOLOGY

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

SHARPIN is a component of the NF-κB-activating linear ubiquitin chain assembly complex

(2011) Fuminori Tokunaga et al.   NATURE

SHARPIN forms a linear ubiquitin ligase complex regulating NF-κB activity and apoptosis

(2011) Fumiyo Ikeda et al.   NATURE

Linear ubiquitination prevents inflammation and regulates immune signalling

(2011) Björn Gerlach et al.   NATURE

Functional analysis of the BMP9 response of ALK1 mutants from HHT2 patients: a diagnostic tool for novel ACVRL1 mutations

(2010) N. Ricard et al.   BLOOD

Update on molecular diagnosis of hereditary hemorrhagic telangiectasia

(2010) Jennifer Richards-Yutz et al.   HUMAN GENETICS

Signaling by members of the TGF-β family in vascular morphogenesis and disease

(2010) Evangelia Pardali et al.   TRENDS IN CELL BIOLOGY

Specific Recognition of Linear Ubiquitin Chains by NEMO Is Important for NF-κB Activation

(2009) Simin Rahighi et al.   CELL

TGF-β Superfamily Signaling in Embryonic Development and Homeostasis

(2009) Mary Y. Wu et al.   DEVELOPMENTAL CELL

Involvement of linear polyubiquitylation of NEMO in NF-κB activation

(2009) Fuminori Tokunaga et al.   NATURE CELL BIOLOGY