The HIF and other quandaries in VHL disease

Modeling Renal Cell Carcinoma in Mice: Bap1 and Pbrm1 Inactivation Drive Tumor Grade

(2017) Yi-Feng Gu et al.   Cancer Discovery

The SWI/SNF Protein PBRM1 Restrains VHL-Loss-Driven Clear Cell Renal Cell Carcinoma

(2017) Amrita M. Nargund et al.   Cell Reports

Formation of Renal Cysts and Tumors inVhl/Trp53-Deficient Mice Requires HIF1α and HIF2α

(2016) Désirée Schönenberger et al.   CANCER RESEARCH

A Small-Molecule Antagonist of HIF2α Is Efficacious in Preclinical Models of Renal Cell Carcinoma

(2016) Eli M. Wallace et al.   CANCER RESEARCH

Novel insights into the polycythemia–paraganglioma–somatostatinoma syndrome

(2016) Roland Därr et al.   ENDOCRINE-RELATED CANCER

Targeting renal cell carcinoma with a HIF-2 antagonist

(2016) Wenfang Chen et al.   NATURE

On-target efficacy of a HIF-2α antagonist in preclinical kidney cancer models

(2016) Hyejin Cho et al.   NATURE

pVHL suppresses kinase activity of Akt in a proline-hydroxylation-dependent manner

(2016) J. Guo et al.   SCIENCE

Hypoxia-Inducible Factor 2α Mutation-Related Paragangliomas Classify as Discrete Pseudohypoxic Subcluster

(2016) Stephanie M.J. Fliedner et al.   NEOPLASIA

Genome-Wide Profiling of TRACK Kidneys Shows Similarity to the Human ccRCC Transcriptome

(2015) L. Fu et al.   MOLECULAR CANCER RESEARCH

Frequent EPAS1/HIF2α exons 9 and 12 mutations in non-familial pheochromocytoma

(2014) Jenny Welander et al.   ENDOCRINE-RELATED CANCER

The multifaceted von Hippel-Lindau tumour suppressor protein

(2014) Claire M. Robinson et al.   FEBS LETTERS

The role of HIF1α in renal cell carcinoma tumorigenesis

(2014) Lorraine J. Gudas et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Paraganglioma and phaeochromocytoma: from genetics to personalized medicine

(2014) Judith Favier et al.   Nature Reviews Endocrinology

Polycythemia and Paraganglioma With a Novel Somatic HIF2A Mutation in a Male

(2014) H. Toyoda et al.   PEDIATRICS

Prospective study assessing hypoxia-related proteins as markers for the outcome of treatment with sunitinib in advanced clear-cell renal cell carcinoma

(2013) J. Garcia-Donas et al.   ANNALS OF ONCOLOGY

Novel HIF2A mutations disrupt oxygen sensing, leading to polycythemia, paragangliomas, and somatostatinomas

(2013) C. Yang et al.   BLOOD

Activation of HIF2  in Kidney Proximal Tubule Cells Causes Abnormal Glycogen Deposition but not Tumorigenesis

(2013) L. Fu et al.   CANCER RESEARCH

Combined mutation ofVhlandTrp53causes renal cysts and tumours in mice

(2013) Joachim Albers et al.   EMBO Molecular Medicine

In vivo and in vitro oncogenic effects of HIF2A mutations in pheochromocytomas and paragangliomas

(2013) Rodrigo A Toledo et al.   ENDOCRINE-RELATED CANCER

Tumoral EPAS1 (HIF2A) mutations explain sporadic pheochromocytoma and paraganglioma in the absence of erythrocytosis

(2013) Iñaki Comino-Méndez et al.   HUMAN MOLECULAR GENETICS

First Report of Bilateral Pheochromocytoma in the Clinical Spectrum ofHIF2A-Related Polycythemia-Paraganglioma Syndrome

(2013) David Taïeb et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Mosaicism inHIF2A-Related Polycythemia-Paraganglioma Syndrome

(2013) Alexandre Buffet et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

HIF-1 mediates metabolic responses to intratumoral hypoxia and oncogenic mutations

(2013) Gregg L. Semenza   JOURNAL OF CLINICAL INVESTIGATION

New Syndrome of Paraganglioma and Somatostatinoma Associated With Polycythemia

(2013) Karel Pacak et al.   JOURNAL OF CLINICAL ONCOLOGY

Comprehensive molecular characterization of clear cell renal cell carcinoma

(2013)   NATURE

A novel EPAS1/HIF2A germline mutation in a congenital polycythemia with paraganglioma

(2012) Felipe R. Lorenzo et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

SomaticHIF2AGain-of-Function Mutations in Paraganglioma with Polycythemia

(2012) Zhengping Zhuang et al.   NEW ENGLAND JOURNAL OF MEDICINE

High-resolution genome-wide mapping of HIF-binding sites by ChIP-seq

(2011) J. Schodel et al.   BLOOD

Generation of a Mouse Model of Von Hippel-Lindau Kidney Disease Leading to Renal Cancers by Expression of a Constitutively Active Mutant of HIF1 

(2011) L. Fu et al.   CANCER RESEARCH

Hallmarks of Cancer: The Next Generation

(2011) Douglas Hanahan et al.   CELL

Chromosome 14q loss defines a molecular subtype of clear-cell renal cell carcinoma associated with poor prognosis

(2011) Federico A Monzon et al.   MODERN PATHOLOGY

Loss of JAK2 regulation via a heterodimeric VHL-SOCS1 E3 ubiquitin ligase underlies Chuvash polycythemia

(2011) Ryan C Russell et al.   NATURE MEDICINE

Genetic and Functional Studies Implicate HIF1  as a 14q Kidney Cancer Suppressor Gene

(2011) C. Shen et al.   Cancer Discovery

ZUR FRAGE DER ANGIOMATOSIS RETINae UND IHRER HIRNKOMPLIKATIONEN

(2009) ARVID LINDAU   ACTA OPHTHALMOLOGICA

Patterns of Gene Expression and Copy-Number Alterations in von-Hippel Lindau Disease-Associated and Sporadic Clear Cell Carcinoma of the Kidney

(2009) R. Beroukhim et al.   CANCER RESEARCH

HIF-2  maintains an undifferentiated state in neural crest-like human neuroblastoma tumor-initiating cells

(2009) A. Pietras et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

von Hippel-Lindau tumor suppressor gene-dependent mRNA stabilization of the survival factor parathyroid hormone-related protein in human renal cell carcinoma by the RNA-binding protein HuR

(2008) Sabrina Danilin et al.   CARCINOGENESIS

The von Hippel-Lindau Tumor Suppressor Protein and Egl-9-Type Proline Hydroxylases Regulate the Large Subunit of RNA Polymerase II in Response to Oxidative Stress

(2008) O. Mikhaylova et al.   MOLECULAR AND CELLULAR BIOLOGY

PHD2Mutation and Congenital Erythrocytosis with Paraganglioma

(2008) Charline Ladroue et al.   NEW ENGLAND JOURNAL OF MEDICINE