| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Farzad Jamshidi, Lola Lozano, Budd Tucker, Jean Andorf, Elliott Sohn, Edwin Stone, Andrew Groves, Yousef Zakharia, H Culver Boldt, Elaine Binkle. Belzutifan in Individuals with von Hippel-Lindau Retinal Hemangioblastomas: Institutional Experience and Review of the Literature. Ocular oncology and pathology. vol 10. issue 3. 2024-09-03. PMID:39224523. |
the systemic hif-2 alpha inhibitor, belzutifan, has been approved for use in patients with von hippel-lindau disease (vhl)-associated renal cell carcinoma, central nervous system (cns) hemangioblastomas, and pancreatic neuroendocrine tumors. |
2024-09-03 |
2024-09-05 |
Not clear |
| Ryugo Teranishi, Tsuyoshi Takahashi, Yukinori Kurokawa, Takuro Saito, Kazuyoshi Yamamoto, Kota Momose, Kotaro Yamashita, Koji Tanaka, Tomoki Makino, Kiyokazu Nakajima, Hidetoshi Eguchi, Yuichiro Dok. Pimitespib, a Novel Heat Shock Protein 90 Inhibitor, Is Effective in Treating Renal Cell Carcinoma by Anti-angiogenetic Signaling. Anticancer research. vol 44. issue 8. 2024-07-26. PMID:39060043. |
hypoxia-inducible factors 1 and 2 alpha (hif1α and hif2α) accumulate in ccrcc with dysfunctional vhl and up-regulate the vascular endothelial growth factor (vegf) pathway and tumor angiogenesis. |
2024-07-26 |
2024-07-29 |
Not clear |
| Bryan J Neth, Mason J Webb, Jessica White, Joon H Uhm, Pavel N Pichurin, Ugur Sene. Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience. Journal of neuro-oncology. 2023-07-14. PMID:37450072. |
belzutifan is a selective inhibitor of hypoxia-inducible factor 2 alpha (hif-2a) that has emerged as a targeted therapy option for von hippel-lindau (vhl) syndrome-associated tumors with recent fda approval. |
2023-07-14 |
2023-08-14 |
Not clear |
| Nitin H Shirole, William G Kaeli. von-Hippel Lindau and Hypoxia-Inducible Factor at the Center of Renal Cell Carcinoma Biology. Hematology/oncology clinics of North America. 2023-06-03. PMID:37270382. |
the vhl protein, pvhl, earmarks the alpha subunits of the hif transcription factor for destruction in an oxygen-dependent manner. |
2023-06-03 |
2023-08-14 |
Not clear |
| Lucie Sochorcova, Katarina Hlusickova Kapralova, Jana Fialova Kucerova, Dagmar Pospisilova, Daniela Prochazkova, Ondrej Jahoda, Simona Kurekova, Barbora Kralova, Martina Divoka, Jana Navratilova, Jirina Manakova, Eva Kriegova, Karel Indrak, Edgar Faber, Vladimir Divoky, Monika Horvathov. Elevated erythroferrone distinguishes erythrocytosis with inherited defects in oxygen-sensing pathway from primary familial and congenital polycythaemia. British journal of haematology. 2023-05-29. PMID:37246471. |
causative mutations in erythropoietin receptor (epor), hypoxia-inducible factor 2 alpha (hif2a) or von hippel-lindau (vhl) genes were detected in nine patients, including a novel p.a421cfs*4 epor and a homozygous intronic c.340+770t>c vhl mutation. |
2023-05-29 |
2023-08-14 |
Not clear |
| Rodrigo A Toledo, Camilo Jimenez, Gustavo Armaiz-Pena, Carlota Arenillas, Jaume Capdevila, Patricia L M Dahi. Hypoxia Inducible factor 2 alpha (HIF2α) inhibitors: targeting genetically driven tumor hypoxia. Endocrine reviews. 2022-10-27. PMID:36301191. |
tumors driven by deficiency of the vhl gene product, which is involved in degradation of the hypoxia inducible factor subunit 2 alpha (hif2α), are natural candidates for targeted inhibition of this pathway. |
2022-10-27 |
2023-08-14 |
Not clear |
| Roberto Iacovelli, Daniela Arduini, Chiara Ciccarese, Francesco Pierconti, Alessandro Strusi, Geny Piro, Carmine Carbone, Nazario Foschi, Gennaro Daniele, Giampaolo Tortor. Targeting hypoxia-inducible factor pathways in sporadic and Von Hippel-Lindau syndrome-related kidney cancers. Critical reviews in oncology/hematology. 2022-06-21. PMID:35728738. |
this review describes the available evidence supporting the use of the small-molecule hif-2 alpha inhibitor, belzutifan (mk-6482), as well as other new anti-hif molecules that have demonstrated significant efficacy in vhl disease-related rccs as well as for sporadic rcc that has progressed after the use of ici-based combinations. |
2022-06-21 |
2023-08-14 |
Not clear |
| Vera M Todd, Lawrence A Vecchi, Miranda E Clements, Katherine P Snow, Cayla D Ontko, Lauren Himmel, Christopher Pinelli, Marjan Rafat, Rachelle W Johnso. Hypoxia inducible factor signaling in breast tumors controls spontaneous tumor dissemination in a site-specific manner. Communications biology. vol 4. issue 1. 2021-10-23. PMID:34556788. |
to better understand the contributions of hypoxia inducible factor 1 alpha (hif1α), hif2α, and general hif pathway activation in metastasis, we employ a pymt-driven spontaneous murine mammary carcinoma model with mammary specific deletion of hif1α, hif2α, or von hippel-lindau factor (vhl) using the cre-lox system. |
2021-10-23 |
2023-08-13 |
mouse |
| Shivannah S Chiatar, Ogechukwu P Eze, Alan R Schoenfel. Expression of VHL Causes Three-Dimensional Morphological Changes in Renal Cells Indicative of Proximal Tubule Differentiation. Cell & developmental biology. vol 2. 2021-10-21. PMID:24308012. |
since vhl is a key member of an ubiquitin e3 ligase complex whose best known target is hypoxia-inducible factor alpha (hif-α), we looked at the effects of hif-α expression on cell morphology. |
2021-10-21 |
2023-08-12 |
Not clear |
| Paulo Afonso de Carvalho, Murilo Bonatelli, Maurício Dener Cordeiro, Rafael Ferreira Coelho, Sabrina Reis, Miguel Srougi, Willian Carlos Nahas, Celine Pinheiro, Katia Ramos Moreira Leit. MCT1 expression is independently related to shorter cancer-specific survival in clear cell renal cell carcinoma. Carcinogenesis. 2021-10-20. PMID:34668521. |
clear cell renal cell carcinoma (ccrcc) has been considered a metabolic disease, with loss of von hippel-lindau (vhl) gene and consequent overexpression of hypoxia-inducible factor 1 alpha (hif-1α), which is central for tumor development and progression. |
2021-10-20 |
2023-08-13 |
Not clear |
| Xianxian Li, Shanshan Zhang, Yongtao Zhang, Pengchao Liu, Mian Li, Yanqin Lu, Jinxiang Ha. Myoblast differentiation of C2C12 cell may related with oxidative stress. Intractable & rare diseases research. vol 10. issue 3. 2021-09-03. PMID:34466339. |
the expression of hypoxia inducible factor1-alpha (hif1-α), hypoxia inducible factor1-beta (hif1-β), von hippel-lindau (vhl), lysyl oxidase (lox), egl-9 family hypoxia-inducible factor 1 (egln1), proline 4-hydroxylase alpha 1 (p4ha1) and heme oxygenase-1 (homx1) in the process of myoblast differentiation was verified by |
2021-09-03 |
2023-08-13 |
Not clear |
| Joshua A Ronen, Meredith Gavin, Misty D Ruppert, Alan N Peiri. Glycemic Disturbances in Pheochromocytoma and Paraganglioma. Cureus. vol 11. issue 4. 2020-09-30. PMID:31275775. |
severe hypoglycemia may also occur with inherited diseases linked to pheochromocytoma such as von hippel-lindau (vhl) disease as well as predominantly epinephrine-producing tumors, given some of the dramatic downstream effects of alpha and beta adrenoceptor agonization. |
2020-09-30 |
2023-08-13 |
Not clear |
| Christ Ordookhanian, Paul E Kaloostian, Samer S Ghostine, Philippe E Spiess, Arnold B Etam. Management Strategies and Outcomes for VHL-related Craniospinal Hemangioblastomas. Journal of kidney cancer and VHL. vol 4. issue 3. 2020-09-29. PMID:28868236. |
it is thought that loss of tumor suppressor function of the vhl gene results in stabilization of hypoxia-inducible factor alpha with downstream activation of cellular proliferative and angiogenic genes that promote tumorigenesis. |
2020-09-29 |
2023-08-13 |
Not clear |
| Tanja Eberhart, Miriam J Schönenberger, Katharina M Walter, Khanichi N Charles, Phyllis L Faust, Werner J Kovac. Peroxisome-Deficiency and HIF-2α Signaling Are Negative Regulators of Ketohexokinase Expression. Frontiers in cell and developmental biology. vol 8. 2020-09-28. PMID:32733884. |
hypoxia or von hippel-lindau (vhl) tumor suppressor loss leads to the stabilization of hypoxia-inducible factors alpha (hif-1α and hif-2α) and the activation of their signaling to mediate adaptive responses. |
2020-09-28 |
2023-08-13 |
Not clear |
| Claire M Robinson, Betty P K Poon, Yoshihito Kano, Fred G Pluthero, Walter H A Kahr, Michael Oh. A Hypoxia-Inducible HIF1-GAL3ST1-Sulfatide Axis Enhances ccRCC Immune Evasion via Increased Tumor Cell-Platelet Binding. Molecular cancer research : MCR. vol 17. issue 11. 2020-07-28. PMID:31427440. |
ccrcc is characterized most frequently by inactivation of vhl tumor suppressor protein that mediates degradation of the alpha subunit of the hypoxia-inducible factor (hif) transcription factor family. |
2020-07-28 |
2023-08-13 |
Not clear |
| Yao-Hui Gao, Zhao-Xia Wu, Li-Qi Xie, Cai-Xia Li, Yu-Qin Mao, Yan-Tao Duan, Bing Han, San-Feng Han, Yun Yu, Hao-Jie Lu, Peng-Yuan Yang, Tian-Rui Xu, Jing-Lin Xia, Guo-Qiang Chen, Li-Shun Wan. VHL deficiency augments anthracycline sensitivity of clear cell renal cell carcinomas by down-regulating ALDH2. Nature communications. vol 8. 2018-11-19. PMID:28643803. |
furthermore, vhl regulates aldh2 expression by directly binding the promoter of -130 bp to -160 bp to activate the transcription of hepatocyte nuclear factor 4 alpha (hnf-4α). |
2018-11-19 |
2023-08-13 |
Not clear |
| Luke J Fulcher, Thomas Macartney, Polyxeni Bozatzi, Annika Hornberger, Alejandro Rojas-Fernandez, Gopal P Sapkot. An affinity-directed protein missile system for targeted proteolysis. Open biology. vol 6. issue 10. 2017-12-12. PMID:27784791. |
the von hippel-lindau (vhl) protein serves to recruit the hypoxia-inducible factor alpha (hif1α) protein under normoxia to the cul2 e3 ubiquitin ligase for its ubiquitylation and degradation through the proteasome. |
2017-12-12 |
2023-08-13 |
human |
| Giada Zurlo, Jianping Guo, Mamoru Takada, Wenyi Wei, Qing Zhan. New Insights into Protein Hydroxylation and Its Important Role in Human Diseases. Biochimica et biophysica acta. vol 1866. issue 2. 2017-06-15. PMID:27663420. |
a classical example of this modification is hypoxia inducible factor alpha (hif-α) prolyl hydroxylation, which affects hif-α protein stability via the von-hippel lindau (vhl) tumor suppressor pathway, a cullin 2-based e3 ligase adaptor protein frequently mutated in kidney cancer. |
2017-06-15 |
2023-08-13 |
human |
| Naoto Kuroda, Yoshiko Agatsuma, Masato Tamura, Petr Martinek, Ondrej Hes, Michal Micha. Sporadic renal hemangioblastoma with CA9, PAX2 and PAX8 expression: diagnostic pitfall in the differential diagnosis from clear cell renal cell carcinoma. International journal of clinical and experimental pathology. vol 8. issue 2. 2016-02-11. PMID:25973115. |
immunohistochemically, neoplastic cells showed diffuse positivity for inhibin-alpha, s-100 protein, vimentin, ca9, pax2 and pax8, but negativity for cytokeratin cam5.2, alpha smooth muscle actin, melanosome, melan a, tfe3 and cathepsin k. in genetic analyses, this tumor showed no changes of vhl gene mutation, hypermethylation and loss of heterozygosity of chromosome 3p. |
2016-02-11 |
2023-08-13 |
Not clear |
| Katharina M Walter, Miriam J Schönenberger, Martin Trötzmüller, Michael Horn, Hans-Peter Elsässer, Ann B Moser, Miriam S Lucas, Tobias Schwarz, Philipp A Gerber, Phyllis L Faust, Holger Moch, Harald C Köfeler, Wilhelm Krek, Werner J Kovac. Hif-2α promotes degradation of mammalian peroxisomes by selective autophagy. Cell metabolism. vol 20. issue 5. 2015-09-04. PMID:25440060. |
low oxygen tension or von hippel-lindau (vhl) tumor suppressor loss is known to stabilize hypoxia-inducible factors alpha (hif-1α and hif-2α) to mediate adaptive responses, but it remains unknown if peroxisome homeostasis and metabolism are interconnected with hif-α signaling. |
2015-09-04 |
2023-08-13 |
mouse |