نتایج جستجو برای: mpl mutation

تعداد نتایج: 292840  

Journal: :Blood 2007
Ronan Chaligné Chloé James Carole Tonetti Rodolphe Besancenot Jean Pierre Le Couédic Fanny Fava Fréderic Mazurier Isabelle Godin Karim Maloum Frédéric Larbret Yann Lécluse William Vainchenker Stéphane Giraudier

The MPL (W515L and W515K) mutations have been detected in granulocytes of patients suffering from certain types of primitive myelofibrosis (PMF). It is still unknown whether this molecular event is also present in lymphoid cells and therefore potentially at the hematopoietic stem cell (HSC) level. Toward this goal, we conducted MPL genotyping of mature myeloid and lymphoid cells and of lymphoid...

Journal: :Blood 2015
Clemens Stockklausner Anne-Christine Klotter Nicole Dickemann Isabelle N Kuhlee Christin M Duffert Carolin Kerber Niels H Gehring Andreas E Kulozik

The interaction between thrombopoietin (THPO) and its receptor c-Mpl regulates downstream cytokine signaling and platelet homeostasis. Hereditary mutations of c-Mpl can either result in loss-of-function and thrombocytopenia or in gain-of-function and thrombocythemia (HT), and are important models to analyze the mechanism of c-Mpl activity. We have analyzed the effect of the c-Mpl P106L gain-of-...

Journal: :Blood 2008
Roberto Pozner Mirta Schattner

Thrombopoietin (TPO) and its receptor c-Mpl are the primary regulators of megakaryocytopoiesis and play a critical role in hematopoietic stem cell biology. Upon TPO binding, c-Mpl facilitates tyrosine (Y) phosphorylation of cytoplasmic signaling proteins and activation of several pathways that control cellular proliferation, megakaryocyte development, and survival. Turning off the TPO signal is...

2014
Zhiyuan Wu Yunqing Zhang Xinju Zhang Xiao Xu Zhihua Kang Shibao Li Chen Zhang Bing Su Ming Guan

A multiplex snapback primer system was developed for the simultaneous detection of JAK2 V617F and MPL W515L/K mutations in Philadelphia chromosome- (Ph-) negative myeloproliferative neoplasms (MPNs). The multiplex system comprises two snapback versus limiting primer sets for JAK2 and MPL mutation enrichment and detection, respectively. Linear-After exponential (LATE) PCR strategy was employed f...

Journal: :Blood 2004
Jianmin Ding Hirokazu Komatsu Atsushi Wakita Miyuki Kato-Uranishi Masato Ito Atsushi Satoh Kazuya Tsuboi Masakazu Nitta Hiroshi Miyazaki Shinsuke Iida Ryuzo Ueda

One Japanese pedigree of familial essential thrombocythemia (FET) inherited in an autosomal-dominant manner is presented. A unique point mutation, serine 505 to asparagine 505 (Ser505Asn), was identified in the transmembrane domain of the c-MPL gene in all of the 8 members with thrombocythemia, but in none of the other 8 unaffected members in this FET family. The Ba/F3 cells expressing the muta...

2001
Meenakshi Gaur George J. Murphy Frederic J. deSauvage Andrew D. Leavitt

Mpl is the thrombopoietin (TPO) receptor. The current molecular understanding of how Mpl activation stimulates proliferation of megakaryocyte-lineage cells is based largely on the engineered expression of Mpl in nonmegakaryocyte-lineage cell lines. However, the relevance of these findings to Mpl signaling in primary megakaryocyte-lineage cells remains largely unknown. Therefore, a system was de...

Journal: :Blood 2005
Philipp S Goerttler Cordula Steimle Edith März Peter L Johansson Björn Andreasson Martin Griesshammer Heinz Gisslinger Hermann Heimpel Heike L Pahl

Recently, a Jak2V617F mutation has been described in the vast majority of patients with polycythemia vera (PV) as well as in subsets of patients with essential thrombocythemia (ET) and idiopathic myelofibrosis (IMF). The question arises whether this mutation is observed in those patients with ET and IMF who have also displayed previously described molecular markers, notably the ability to form ...

Journal: :Blood 2014
Elisa Rumi Daniela Pietra Cristiana Pascutto Paola Guglielmelli Alejandra Martínez-Trillos Ilaria Casetti Dolors Colomer Lisa Pieri Marta Pratcorona Giada Rotunno Emanuela Sant'Antonio Marta Bellini Chiara Cavalloni Carmela Mannarelli Chiara Milanesi Emanuela Boveri Virginia Ferretti Cesare Astori Vittorio Rosti Francisco Cervantes Giovanni Barosi Alessandro M Vannucchi Mario Cazzola

We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515) mutation, and 53 (8.6%) had nonmutated JAK2, CALR, and MPL (so-called triple-...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1999
K Ihara

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare disorder expressed in infancy and characterized by isolated thrombocytopenia and megakaryocytopenia with no physical anomalies. Our previous hematological analysis indicated similarities between human CAMT and murine c-mpl (thrombopoietin receptor) deficiency. Because the c-mpl gene was considered as one of the candidate genes for thi...

Journal: :Blood 2016
Marito Araki Yinjie Yang Nami Masubuchi Yumi Hironaka Hiraku Takei Soji Morishita Yoshihisa Mizukami Shin Kan Shuichi Shirane Yoko Edahiro Yoshitaka Sunami Akimichi Ohsaka Norio Komatsu

Recurrent somatic mutations of calreticulin (CALR) have been identified in patients harboring myeloproliferative neoplasms; however, their role in tumorigenesis remains elusive. Here, we found that the expression of mutant but not wild-type CALR induces the thrombopoietin (TPO)-independent growth of UT-7/TPO cells. We demonstrated that c-MPL, the TPO receptor, is required for this cytokine-inde...

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