Abstract:
PURPOSE OF REVIEW:There are three kindlin family members in vertebrates, which have high-sequence homology and a common organization signature with a C-terminal 4.1, ezrin, radixin, moesin (FERM) domain bisected by a pleckstrin-homology domain. Although the cell and tissue distributions of the three kindlins differ, there is a consistent and close interrelationship between kindlins and integrins, and alterations of kindlin expression affect integrin-dependent functions. However, in-vivo data on the functions of the kindlins and their mechanisms of action have been lacking. RECENT FINDINGS:Recent studies have shown that deficiencies of each of the three kindlins result in profound and distinct phenotypes, ranging from skin and intestinal defects (kindlin-1), embryonic lethality due to cardiac developmental problems (kindlin-2), to marked abnormalities in platelet, leukocyte and erythrocyte function (kindlin-3). A human disease characterized by bleeding, frequent infections and osteopetrosis has now been attributed to mutations in the gene encoding for kindlin-3. These defects are consistent with recent data showing that kindlins control integrin activation and function. SUMMARY:The three members of the kindlin family have now been implicated as essential regulators of integrin function in individual cells and in whole organisms.
journal_name
Curr Opin Hematoljournal_title
Current opinion in hematologyauthors
Plow EF,Qin J,Byzova Tdoi
10.1097/MOH.0b013e32832ea389subject
Has Abstractpub_date
2009-09-01 00:00:00pages
323-8issue
5eissn
1065-6251issn
1531-7048journal_volume
16pub_type
杂志文章,评审abstract:PURPOSE OF REVIEW:Over the last years, long non-coding RNAs (lncRNAs) have emerged as putative regulators of malignant hematopoietic development. Here, we review recent literature on the involvement of lncRNAs in leukemia, including their role in driving or sustaining disease and their potential impact on diagnosis, cl...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000354
更新日期:2017-07-01 00:00:00
abstract:PURPOSE OF REVIEW:To examine the current literature regarding retrievable inferior vena cava filters and to discuss the appropriate indications for their clinical use. RECENT FINDINGS:Permanent filters have been shown to be effective, but have a number of long-term complications such as filter thrombosis or migration....
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000239707.63168.ee
更新日期:2006-09-01 00:00:00
abstract::Allogeneic barriers to transfusion are caused by differences between those portions of the donor and recipient genomes that define the antigenicity and immune response to the transfused cells. Historically, a blood group antigen was identified when an immune response (alloantibody) was detected by hemagglutination in ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200203000-00012
更新日期:2002-03-01 00:00:00
abstract::The aim of gene therapy for hemophilia is the stable introduction and expression of a gene encoding functional blood coagulation factor VIII or IX. Although there are as yet no published studies demonstrating long-term expression of therapeutic levels in large animal models of the disease, there have been several repo...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199809000-00003
更新日期:1998-09-01 00:00:00
abstract::Platelet production is primarily regulated by the thrombopoietic cytokine thrombopoietin (TPO). In most cases thrombopoietin serum levels are determined by the rate of c-mpl receptor-mediated degradation after TPO uptake into platelets and megakaryocytes. The contribution of increased TPO protein synthesis by a transl...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200005000-00003
更新日期:2000-05-01 00:00:00
abstract:PURPOSE OF REVIEW:The identification of regulatory T cells (Tregs) as regulators of immunological self-tolerance has stimulated tremendous interest in the field. Over the past 12 months, new studies have added greatly to our understanding of the role of Tregs in autoimmune disease, details of which are presented here. ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32832a9a01
更新日期:2009-07-01 00:00:00
abstract::Whereas there are general guidelines for acceptable transfusion therapy, optimal transfusion therapy has not been determined for most clinical settings. Recent research has focused on controlled studies of red cell transfusion in specific clinical settings. Better determinations of oxygen delivery and consumption are ...
journal_title:Current opinion in hematology
pub_type: 杂志文章
doi:10.1097/00062752-200111000-00012
更新日期:2001-11-01 00:00:00
abstract::FLT3 is the most frequently mutated gene in cases of acute myelogenous leukemia (AML). About 30 to 35% of patients have either internal tandem duplications (ITDs) in the juxtamembrane domain or mutations in the activating loop of FLT3. FLT3 mutations occur in a broad spectrum of FAB subtypes in adult and pediatric AML...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200207000-00003
更新日期:2002-07-01 00:00:00
abstract:PURPOSE OF REVIEW:Type 1 von Willebrand disease (VWD) is the most common form of VWD, but has remained [corrected] the least well understood. Recent work is changing this situation. This review summarizes recent analysis of the genetic basis of the disease. RECENT FINDINGS:Linkage analysis demonstrates that dominantly...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e32826f4b41
更新日期:2007-09-01 00:00:00
abstract:PURPOSE OF REVIEW:The purpose of this review is to describe the physiopathological and therapeutic aspects of neutrophilic dermatosis, taking into account their most frequent associated conditions. RECENT FINDINGS:In autoinflammatory syndromes featuring neutrophilic dermatosis, the role of interleukin-1 and tumor necr...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000100
更新日期:2015-01-01 00:00:00
abstract::Disorders of the erythrocyte membrane, such as hereditary spherocytosis, hereditary elliptocytosis, and hereditary pyropoikilocytosis, are characterized by heterogeneity in their clinical and laboratory manifestations. Recent work has shown that these disorders also exhibit marked genetic heterogeneity. Multiple genet...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199704020-00009
更新日期:1997-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Glycogen storage disease Ib (GSD Ib) is characterized by hepatomegaly, hypoglycemia, neutropenia, enterocolitis and recurrent bacterial infections. It is attributable to mutations in G6PT1, the gene for the glucose-6-phosphate transporter responsible for transport of glucose into the endoplasmic retic...
journal_title:Current opinion in hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1097/MOH.0000000000000474
更新日期:2019-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Iron homeostasis and erythropoiesis regulate each other to ensure optimal delivery of oxygen and iron to cells and tissues. Defining the mechanisms of this crosstalk is important for understanding the pathogenesis of common conditions associated with disordered iron metabolism and erythropoiesis. REC...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000132
更新日期:2015-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Mutations in the Endoglin (Eng) gene, an auxiliary receptor in the transforming growth factor beta (TGFβ)-superfamily signaling pathway, are responsible for the human vascular disorder hereditary hemorrhagic telangiectasia (HHT) type 1, characterized in part by blood vessel enlargement. A growing body...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000419
更新日期:2018-05-01 00:00:00
abstract:PURPOSE OF REVIEW:New discoveries focused on mitochondrial metabolism and gene silencing and their regulation by the sirtuin family of protein deacetylases is stimulating new ideas on how to improve geriatric medicine. Information about sertuins in stem cell biology is scarce. We consider recent information on sirtuin ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283043819
更新日期:2008-07-01 00:00:00
abstract::Acute chest syndrome (ACS) is the presence of a new pulmonary infiltrate in combination with fever or respiratory symptoms in a patient with sickle cell disease. ACS is the leading cause of death in sickle cell disease, and many patients suffer from multiple, severe episodes. Age has a striking effect on the clinical ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199803000-00002
更新日期:1998-03-01 00:00:00
abstract::This overview concentrates on familial and congenital polycythemias in the context of other polycythemic disorders, with emphasis on those with established molecular lesions. Recent advances in the regulation of erythropoiesis, as they may relate to polycythemic states, are discussed as a background for those well-def...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-199903000-00008
更新日期:1999-03-01 00:00:00
abstract:PURPOSE OF REVIEW:Neutrophils priming has been long studied in vitro. Recent studies describe it in vivo. In pathophysiological conditions, complex, heterogeneous characteristics of priming are described in the last few years. RECENT FINDINGS:Priming can occur systemically when insults such as sepsis or trauma result ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000471
更新日期:2019-01-01 00:00:00
abstract:PURPOSE OF REVIEW:Clonal heterogeneity is a significant obstacle to successful treatment of patients with acute myeloid leukemia (AML). Here, we review new advances in the understanding of genetic heterogeneity in AML using single-cell DNA-sequencing technology. RECENT FINDINGS:New genomics and immunologic discovery t...
journal_title:Current opinion in hematology
pub_type: 杂志文章
doi:10.1097/MOH.0000000000000626
更新日期:2021-01-01 00:00:00
abstract::Hematopoietic stem cells, defined as cells with extensive self-renewal and pluripotent differentiation potential, represent a minor population of adult bone marrow (< 1 in 10(4) to 10(5) nucleated marrow cells). Recent advances in cell surface phenotype analysis and separation technology have permitted enrichment of h...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:
更新日期:1994-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic stem cells are functionally heterogeneous even when isolated as phenotypically homogenous populations. How this heterogeneity is generated is incompletely understood. Several models have been formulated to explain the generation of diversity. All of these assume the existence of a single...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/01.moh.0000231421.00407.65
更新日期:2006-07-01 00:00:00
abstract::The advent of safe and practical means to correct, enhance or protect blood cells at the genetic level offers tantalizing therapeutic perspectives. At present, gene delivery using a replication-defective retrovirus is the most efficient method to stably transduce hematopoietic cells. The successful adaptation of retro...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200011000-00008
更新日期:2000-11-01 00:00:00
abstract:PURPOSE OF REVIEW:Obesity has become a worldwide epidemic that is driving increased morbidity and mortality from thrombotic disorders such as myocardial infarction, stroke, and venous thromboembolism. Effective prevention and treatment of thrombosis in obese patients is limited by an incomplete understanding of the und...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3283634443
更新日期:2013-09-01 00:00:00
abstract:PURPOSE OF REVIEW:This article summarizes recent insights into the pathophysiology of immune thrombocytopenic purpura, a disorder in which autoantibodies against cell-specific glycoproteins (GPIIb-IIIa, GPIb-IX and others) accelerate platelet destruction. RECENT FINDINGS:Autoantibodies are produced by a limited number...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3282ba5552
更新日期:2007-09-01 00:00:00
abstract::Platelet aggregation, a process mediated by GP IIb-IIIa, is responsible for the occlusive events in thrombosis: indeed, GP IIb-IIIa antagonists are effective in blocking arterial thrombosis. Recent studies have suggested that activated platelets and platelet thrombi can contribute significantly to the initiation and p...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200309000-00006
更新日期:2003-09-01 00:00:00
abstract::Several methods to model human Ph+ leukemia in laboratory mice are available, including propagation of BCR/ABL-expressing cells in mice, xenotransplantation of primary Ph+ leukemia cells into immunodeficient mice, BCR/ABL transgenic mice, and BCR/ABL retroviral bone marrow transduction and transplantation. Recent stud...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200107000-00008
更新日期:2001-07-01 00:00:00
abstract:PURPOSE OF REVIEW:In addition to its essential role in baseline erythropoiesis, the hormone erythropoietin drives the erythropoietic response to hypoxic stress. A mechanistic understanding of stress erythropoiesis would benefit multiple clinical settings, and may aid in understanding leukemogenesis. RECENT FINDINGS:Th...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0b013e3280de2bf1
更新日期:2007-05-01 00:00:00
abstract::The advent of better diagnostic tools, molecular techniques, and cytokine therapies have come together at the end of the 20th century to provide an improved outlook for patients with autoimmune neutropenia. Severe chronic neutropenia is no longer idiopathic in most cases, and growth factor therapy can be safely offere...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/00062752-200105000-00007
更新日期:2001-05-01 00:00:00
abstract:PURPOSE OF REVIEW:Microtransplantation (MST) has been successfully used to manage patients with hematological malignancies, especially older patients with acute myeloid leukemia (AML). Recent clinical applications and mechanistic studies of MST are reviewed. RECENT FINDINGS:MST improves complete remission rates which ...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000470
更新日期:2018-11-01 00:00:00
abstract:PURPOSE OF REVIEW:Hematopoietic stem cells (HSCs) possess two fundamental characteristics, the capacity for self-renewal and the sustained production of all blood cell lineages. The fine balance between HSC expansion and lineage specification is dynamically regulated by the interplay between external and internal stimu...
journal_title:Current opinion in hematology
pub_type: 杂志文章,评审
doi:10.1097/MOH.0000000000000260
更新日期:2016-07-01 00:00:00