التفاصيل البيبلوغرافية
| العنوان: |
Distinct stem cell myeloproliferative/T lymphoma syndromes induced by ZNF198-FGFR1 and BCR-FGFR1 fusion genes from 8p11 translocations |
| المؤلفون: |
Roumiantsev, Sergei, Krause, Daniela S, Neumann, Carola A, Dimitri, Christopher A, Asiedu, Frances, Cross, Nicholas CP, Van Etten, Richard A |
| المصدر: |
Cancer Cell, vol 5, iss 3 |
| بيانات النشر: |
eScholarship, University of California |
| سنة النشر: |
2004 |
| المجموعة: |
University of California: eScholarship |
| مصطلحات موضوعية: |
Rare Diseases, Stem Cell Research - Nonembryonic - Human, Lymphoma, Hematology, Cancer, Stem Cell Research, Aetiology, 2.1 Biological and endogenous factors, Adaptor Proteins, Signal Transducing, Animals, Bone Marrow, Carrier Proteins, Chromosome Breakage, Chromosomes, Human, Pair 8, DNA-Binding Proteins, GRB2 Adaptor Protein, Humans, Leukemia, Myelogenous, Chronic, BCR-ABL Positive, T-Cell, Mice, Myeloproliferative Disorders, Oncogene Proteins, Phospholipase C gamma, Protein-Tyrosine Kinases |
| جغرافية الموضوع: |
287 - 298 |
| الوصف: |
8p11 myeloproliferative syndrome (EMS) is a hematopoietic stem cell disorder characterized by myeloid hyperplasia and non-Hodgkin's lymphoma with chromosomal translocations fusing several genes, most commonly ZNF198, to fibroblast growth factor receptor-1 (FGFR1). However, patients with BCR-FGFR1 fusion present with typical chronic myeloid leukemia (CML). We demonstrate that ZNF198-FGFR1 induces EMS-like disease in mice, with myeloproliferation and T lymphoma arising from common multipotential progenitors. Mutation of FGFR1 Tyr766 attenuates both myeloid and lymphoid diseases, identifying phospholipase C-gamma1 as a downstream effector. Bcr-FGFR1 binds Grb2 via Bcr Tyr177 and induces CML-like leukemia in mice, whereas Bcr-FGFR1/Y177F lacks Grb2 binding and causes EMS-like disease. These results implicate different signaling pathways originating from both kinase and fusion partner in the pathogenesis of CML and EMS. |
| نوع الوثيقة: |
article in journal/newspaper |
| وصف الملف: |
application/pdf |
| اللغة: |
unknown |
| العلاقة: |
qt5614h8vs; https://escholarship.org/uc/item/5614h8vsTest |
| الإتاحة: |
https://escholarship.org/uc/item/5614h8vsTest |
| حقوق: |
CC-BY |
| رقم الانضمام: |
edsbas.DB6CF4EE |
| قاعدة البيانات: |
BASE |
