The von Hippel–Lindau Tumor Suppressor Protein
نویسندگان
چکیده
منابع مشابه
The von Hippel-Lindau tumor suppressor gene and kidney cancer.
The von Hippel-Lindau tumor suppressor gene (VHL), which resides on chromosome 3p25, is mutated or silenced in >50% of sporadic clear cell renal cell carcinomas. Germ-line VHL mutations give rise to VHL disease, which is characterized by an increased risk of blood vessel tumors (hemangioblastomas) and renal cell carcinomas. In this setting, VHL inactivation gives rise to premalignant renal cyst...
متن کاملThe von Hippel-Lindau tumor suppressor targets to mitochondria.
Subcellular localization of von Hippel-Lindau (VHL) tumor suppressor may clarify its role in tumorigenesis. In rat kidney, we observed a granular cytoplasmic immunostaining of VHL, as seen in human tissues. The green fluorescent protein (GFP)-tagged VHL also appeared as cytoplasmic granules in vitro and was colocalized with a mitochondrion-selective dye. Immunogold electron microscopy localized...
متن کاملRole of the von Hippel-Lindau tumor suppressor protein during neuronal differentiation.
The von Hippel-Lindau (VHL) tumor suppressor protein down-regulates transcription by transcriptional elongation enhanced by antagonizing elongin B and C. Transcriptional regulation is an important control mechanism for embryogenesis and tumorigenesis. The VHL gene and protein are expressed in neuronal cells of the fetal and adult brain. However, the role of the VHL gene in the central nervous s...
متن کاملThe von Hippel-Lindau tumor suppressor protein: roles in cancer and oxygen sensing.
Biallelic inactivation of the von Hippel-Lindau (VHL) tumor suppressor gene is a common event in hereditary (von Hippel- Lindau disease) and sporadic hemangioblastomas and clear-cell renal carcinomas. Germ-line VHL mutations are also linked to some hereditary pheochromocytoma families. The VHL gene product, pVHL, interacts with a number of cellular proteins and is implicated in the control of a...
متن کاملThe von Hippel-Lindau tumor suppressor protein and clear cell renal carcinoma.
Germ line VHL tumor suppressor gene loss-of-function mutations cause von Hippel-Lindau disease, which is associated with an increased risk of central nervous system hemangioblastomas, clear cell renal carcinomas, and pheochromocytomas. Somatic VHL mutations are also common in sporadic clear cell renal carcinomas. The VHL gene product, pVHL, is part of a ubiquitin ligase complex that targets the...
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ژورنال
عنوان ژورنال: Annual Review of Cancer Biology
سال: 2018
ISSN: 2472-3428,2472-3428
DOI: 10.1146/annurev-cancerbio-030617-050527