نتایج جستجو برای: مه cbl

تعداد نتایج: 3107  

Journal: :Hematology 2016
Basem M William Wei An Dan Feng Scott Nadeau Bhopal C Mohapatra Matthew A Storck Vimla Band Hamid Band

OBJECTIVES Mutations in Cbl or Cbl-b gene occur in 10% of myeloproliferative disorder (MPD) patients and are associated with poor prognosis. Hematopoietic Cbl/Cbl-b double knockout (DKO) leads to a disease in mice phenotypically similar to human MPDs. The aim of this study was to evaluate the anti-MPD activity of a clinically safe drug, Fasudil, identified in an in vitro kinase inhibitor as an ...

2014
Sadok Yakoub Nisrine El - Chami Krisztian Kaszas Mouhannad Malek Mohamed El Sirkasi Colin A. Smith Elias Baydoun Eric Tabone Serge N Manié Daniel CL Régnier

The proto-oncoprotein c-Cbl controls several down-regulation signals leading to degradation of tyrosine-kinase receptors (TKR) [1-3] or endocytic trafficking [1,4,5]. It interacts with numerous signalling proteins through their Src homology-2 or -3 (SH2 or SH3) domains [6,7]. Several groups, including ours, have reported an association between down-regulation of apoptosis and c-Cbl. First, onco...

Journal: :The British journal of nutrition 2013
Toby Andrew Raj Gill Irina Gillham-Nasenya Kourosh R Ahmadi

Cobalamin (Cbl) is an essential B vitamin involved in the normal functioning of the nervous system, the formation of key components of blood, DNA synthesis and methylation, and energy production. Physiological levels of Cbl vary greatly within populations, although the basis for this variability remains largely unknown. We conducted a twin study to characterise the basis of variation in plasma ...

Journal: :Blood 1979
C A Hall W H Hitzig P D Green J A Begley

Cobalamin (Cbl) transport was studied in the circulation of a child deficient in transcobalamin II (TC II) after 1.0 mg of cyanocobalamin (CN-Cbl) either intramuscularly or orally. Pertinent observations include the following: (1) There was no detectable TC II in the serum as measured immunologically or by binding capacities in vivo or in vitro. (2) Transcobalamin I (TC I) was present in at lea...

Journal: :The Journal of biological chemistry 2001
M Yokouchi T Kondo A Sanjay A Houghton A Yoshimura S Komiya H Zhang R Baron

The protooncogene c-Cbl has recently emerged as an E3 ubiquitin ligase for activated receptor tyrosine kinases. We report here that c-Cbl also mediates the ubiquitination of another protooncogene, the non-receptor tyrosine kinase c-Src, as well as of itself. The c-Cbl-dependent ubiquitination of Src and c-Cbl requires c-Cbl's RING finger, Src kinase activity, and c-Cbl's tyrosine phosphorylatio...

Journal: :The Journal of clinical investigation 1978
P Youngdahl-Turner L E Rosenberg R H Allen

We have used purified, (125)I-labeled human transcobalamin II (TC II), saturated with cobalamin (Cbl), to study the uptake process for the TC II-Cbl complex by intact normal cultured human skin fibroblasts. We have also investigated the possibility that a defect in one step of this process underlies that inborn error of Cbl metabolism-designated cbl C-in which mutant cells are unable to retain ...

Journal: :Blood 2009
Srinivasa Rao Bandi Christian Brandts Marion Rensinghoff Rebekka Grundler Lara Tickenbrock Gabriele Köhler Justus Duyster Wolfgang E Berdel Carsten Müller-Tidow Hubert Serve Bülent Sargin

Somatic mutations of Kit have been found in leukemias and gastrointestinal stromal tumors. The proto-oncogene c-Cbl negatively regulates Kit and Flt3 by its E3 ligase activity and acts as a scaffold. We recently identified the first c-Cbl mutation in human disease in an acute myeloid leukemia patient, called Cbl-R420Q. Here we analyzed the role of Cbl mutants on Kit-mediated transformation. Coe...

2015
Wei An Scott A. Nadeau Bhopal C. Mohapatra Dan Feng Neha Zutshi Matthew D. Storck Priyanka Arya James E. Talmadge Jane L. Meza Vimla Band Hamid Band

Cbl and Cbl-b are tyrosine kinase-directed RING finger type ubiquitin ligases (E3s) that negatively regulate cellular activation pathways. E3 activity-disrupting human Cbl mutations are associated with myeloproliferative disorders (MPD) that are reproduced in mice with Cbl RING finger mutant knock-in or hematopoietic Cbl and Cbl-b double knockout. However, the role of Cbl proteins in hematopoie...

Journal: :The Journal of biological chemistry 2007
Ning Li Marta Lorinczi Keith Ireton Lisa A Elferink

Lysosomal degradation of the receptor-tyrosine kinase cMet requires receptor ubiquitination by the E3 ubiquitin ligase Cbl followed by clathrin-dependent internalization. A role for Cbl as an adaptor for cMet internalization has been previously reported. However, the requirement for Cbl ubiquitin ligase activity in this process and its mode of recruitment to cMet has yet to be determined. Cbl c...

2012
Inès Ben Ghezala Johan Frederik Berg Arendt Rune Erichsen Jihen Zalfani Henrik Gammelager Trine Frøslev Morten Olsen

PURPOSE The aim of this validation study was to assess the positive predictive value (PPV) of the International Classification of Diseases, 10th revision (ICD-10) diagnosis of vitamin B12 (cobalamin [Cbl]) deficiency anemia in the Danish National Patient Register (DNPR). PATIENTS AND METHODS We identified all patients above 18 years of age recorded in the DNPR with a diagnosis of Cbl deficien...

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