Abstract:
:Fanconi anemia (FA) is an autosomal recessive syndrome characterized by progressive bone marrow failure and cancer predisposition. Eight FA complementation groups have been identified. The FANCA, FANCC, FANCE, FANCF, and FANCG proteins form a nuclear complex required for the monoubiquination of the FANCD2 protein. To investigate the architecture of the FA protein complex, the yeast 2-hybrid system was used to map contact points of the FANCA/FANCG, FANCC/FANCE, and FANCF/FANCG interactions. FANCG was shown to interact with both the amino-terminus of FANCA and the carboxyl-terminal region of FANCF. A FANCG mutant truncated at the carboxyl-terminus retained the ability to interact with FANCA. The interaction between FANCG and FANCF was ablated by a Leu71Pro mutant of FANCG. A central region of FANCE was sufficient for FANCC binding. A Leu554Pro mutant of FANCC failed to interact with FANCE. To further examine complex assembly, the yeast 3-hybrid system was used to investigate the ability of FANCG to act as a molecular bridge in mediating interaction between other FA proteins. FANCG was able to mediate interaction between FANCA and FANCF, as well as between monomers of FANCA. Direct interaction between FANCE and FANCD2 was also demonstrated in the yeast 2-hybrid system. This interaction involving an amino-terminal region of FANCD2 may provide a link between the FA protein complex and its downstream targets.
journal_name
Bloodjournal_title
Bloodauthors
Gordon SM,Buchwald Mdoi
10.1182/blood-2002-11-3517subject
Has Abstractpub_date
2003-07-01 00:00:00pages
136-41issue
1eissn
0006-4971issn
1528-0020pii
2002-11-3517journal_volume
102pub_type
杂志文章相关文献
BLOOD文献大全abstract::Chronic myeloid leukemia is effectively treated with imatinib, but reactivation of BCR-ABL frequently occurs through acquisition of kinase domain mutations. The additional approved ABL tyrosine kinase inhibitors (TKIs) nilotinib and dasatinib, along with investigational TKIs such as ponatinib (AP24534) and DCC-2036, s...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-05-349191
更新日期:2011-11-10 00:00:00
abstract::IL-15 is an important cytokine for the function of the immune system, but the form(s) of IL-15 produced in the human body are not fully characterized. Coexpression of the single-chain IL-15 and the IL-15 receptor alpha (IL-15Rα) in the same cell allows for efficient production, surface display, and eventual cleavage a...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-10-384362
更新日期:2012-07-05 00:00:00
abstract::Endothelial progenitor cells are critically involved in essential biologic processes, such as vascular homeostasis, regeneration, and tumor angiogenesis. Endothelial colony-forming cells (ECFCs) are endothelial progenitor cells with robust proliferative potential. Their profound vessel-forming capacity makes them a pr...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2008-09-181362
更新日期:2009-06-25 00:00:00
abstract::Glycoprotein V (GPV) is a membrane-associated, 82 Kd platelet glycoprotein that is hydrolyzed during thrombin activation to yield 69 Kd fragment. We have developed a rapid and simple method for isolation of the protein from platelet extracts using a combination of gel permeation, anion-exchange, and lectin affinity ch...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-06-15 00:00:00
abstract::Indolent systemic mastocytosis (SM) patients have a varied clinical presentation, ranging from predominantly cutaneous symptoms to recurrent systemic symptoms (eg, flushing, palpitations, dyspepsia, diarrhea, bone pain) that can be severe and potentially life threatening (anaphylaxis). Mastocytosis patients without sk...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2013-01-453183
更新日期:2013-04-18 00:00:00
abstract::Tryptases are serine proteases primarily expressed in mast cells. Normal blood basophils express only trace amounts of the enzyme. However, recent immunohistochemical studies have raised the possibility that neoplastic basophils express significant amounts of tryptase. In this study, tryptase expression was analyzed i...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v98.8.2580
更新日期:2001-10-15 00:00:00
abstract::In previous studies, the prevalence of HLA-linked hemochromatosis, thought to be the most common genetic illness in whites, has been estimated by identifying homozygotes in the population. Because not all homozygotes express the disease phenotypically, the accuracy of these estimates is uncertain. We analyzed the dist...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1995-09-01 00:00:00
abstract::An inhibitor of procoagulant and fibrinolytic enzymes was derived from cabbage seeds by a procedure using acetone precipitation, ion-exchange chromatography, and gel filtration. The cabbage seed inhibitor was a 10-Kd monomeric protein with intrachain disulfide bonds. This preparation prevented clot formation in whole ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-01-01 00:00:00
abstract::Perioperative management of antithrombotic therapy is a situation that occurs frequently and requires consideration of the patient, the procedure, and an expanding array of anticoagulant and antiplatelet agents. Preoperative assessment must address each patient's risk for thromboembolic events balanced against the ris...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2012-05-423228
更新日期:2012-12-06 00:00:00
abstract::The transcription factor c-Maf is extensively involved in the pathophysiology of multiple myeloma (MM), a fatal malignancy of plasma cells. In the present study, affinity chromatography and mass spectrometry were used to identify c-Maf ubiquitination-associated proteins, from which the E3 ligase HERC4 was found to int...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2015-07-658203
更新日期:2016-03-31 00:00:00
abstract::Monocytes are thought to play a role in host resistance to tumor cell growth in animals and humans. In addition, platelets are known to be involved in tumor metastases. To investigate the interaction of these two cell types and their effect on tumor cells, human monocytes and platelets were examined using an in vitro ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1985-05-01 00:00:00
abstract::Ribosomal protein S19 (RPS19) is frequently mutated in Diamond-Blackfan anemia (DBA), a rare congenital hypoplastic anemia. Recent studies have shown that RPS19 expression decreases during terminal erythroid differentiation. Currently no information is available on the subcellular localization of normal RPS19 and the ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2002-12-3878
更新日期:2003-06-15 00:00:00
abstract::Increased levels of endothelin-1 (Et-1), a potent vasoconstrictor, have been correlated with hypertension and neuronal damage in ischemic/reperfusion injury. The presence of polymorphonuclear cells (PMNs) in the brain has been shown to be directly responsible for this observed pathology. To address the question of whe...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1998-11-01 00:00:00
abstract::Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive biologics and drugs, and supportive care. However, management of SAA patients remains challenging, both acutely in addressing the immediate consequences...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2011-12-274019
更新日期:2012-08-09 00:00:00
abstract::Serial erythrocyte volume distribution curves were used to characterize changes in erythrocyte subpopulations following the induction of Heinz body hemolytic anemia in cats. Macrocytes produced in response to hemolysis were observed after loss of reticulum. After recovery from hemolysis, a gradual reduction in mean ce...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1982-08-01 00:00:00
abstract::A defect in erythropoietin (EPO) production has been advocated as being the main cause of anemia presented at time of diagnosis or during treatment by adults with solid tumors. On the basis of this defect, anemic cancer patients, both adults and children, have been treated with recombinant human EPO (rHuEPO). To furth...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1998-09-01 00:00:00
abstract::Bryostatin 1, a macrocyclic lactone isolated from the marine bryozoan Bugula neritina, has demonstrated both antineoplastic activity against the murine P388 leukemia line in vivo and stimulatory activity against mouse and human hematopoietic progenitors. We studied the effects of bryostatin 1 on the growth of human le...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1990-03-15 00:00:00
abstract::Treatment of AL amyloidosis patients with high-dose melphalan chemotherapy followed by autologous peripheral blood stem cell transplantation (HDM/SCT) can produce hematologic complete responses (CRs) and improvement in organ function. To determine whether these responses are accompanied by improvement in quality of li...
journal_title:Blood
pub_type: 临床试验,杂志文章
doi:10.1182/blood-2004-01-0089
更新日期:2004-09-15 00:00:00
abstract::Severe elevation of red blood cell number is often associated with hypertension and thromboembolism resulting in severe cardiovascular complications. However, some individuals such as high altitude dwellers cope well with an increased hematocrit level. We analyzed adaptive mechanisms to excessive erythrocytosis in our...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-01-0283
更新日期:2003-09-15 00:00:00
abstract::In patients with common variable immunodeficiency (CVI), we have previously defined a subgroup of patients (CVIHyper) characterized by decreased numbers of CD4+ lymphocytes in peripheral blood, splenomegaly, and persistent immune activation in vivo, particularly of monocytes/macrophages. To further characterize this h...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1996-01-15 00:00:00
abstract::We report the international experience in outcomes after related and unrelated hematopoietic transplantation for infantile osteopetrosis in 193 patients. Thirty-four percent of transplants used grafts from HLA-matched siblings, 13% from HLA-mismatched relatives, 12% from HLA-matched, and 41% from HLA-mismatched unrela...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2015-01-625541
更新日期:2015-07-09 00:00:00
abstract::The anti-CD33 antibody calicheamicinconjugate gemtuzumab ozogamicin (GO) was used to treat 16 patients with acute promyelocytic leukemia (APL) who had relapsed at the molecular level. Of these patients, 8 were experiencing a first, 5 a second, 2 a third, and 1 a fourth relapse. GO was administered at 6 mg/m2 for 2 dos...
journal_title:Blood
pub_type: 临床试验,杂志文章
doi:10.1182/blood-2004-04-1550
更新日期:2004-10-01 00:00:00
abstract::Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors. We previously described the deficiency of factor H-related proteins CFHR1 and CFHR3 as predisposing factor for aHUS. Here we identify in an extended cohort of 147 aHUS...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2007-09-109876
更新日期:2008-02-01 00:00:00
abstract::Human blood contains 2 populations of dendritic cells (DCs): plasmacytoid and myeloid (mDC). mDCs are subdivided into 3 subsets using the surface markers CD16, CD1c, and BDCA-3. Their role as pathogen sentinels and adjuvant targets was tested by phenotypic and functional analysis. We show that mDC subsets are immature...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-08-038422
更新日期:2007-06-15 00:00:00
abstract::The genes for the hemopoietic growth factors, GM colony-stimulating factor (CSF) and G-CSF have been cloned, and recombinant material is available for both. We tested these recombinant factors for their effects on the blast cells of acute myeloblastic leukemia (AML). Culture methods are available that support both col...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-05-01 00:00:00
abstract::The transcription factor encoded by the mi locus (MITF) is a transcription factor of the basic-helix-loop-helix zipper protein family. Mice of mi/mi genotype express a normal amount of abnormal MITF, whereas mice of tg/tg genotype do not express any MITFs due to the transgene insertional mutation. The effect of normal...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v97.3.645
更新日期:2001-02-01 00:00:00
abstract::Without a prior history of hemorrhagic disease, a 62-year-old man suffered recurrent episodes of bleeding. Solubility of the patient's clot in 5 mol/L urea indicated a problem with fibrin stabilization. The transamidase activity potential of factor XIII, measured by the incorporation of radioactive putrescine into N,N...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-02-01 00:00:00
abstract::Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accou...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2016-10-746933
更新日期:2017-03-23 00:00:00
abstract::The first hematopoietic and endothelial progenitors are derived from a common embryonic precursor termed the hemangioblast. The genetic cascades that regulate the differentiation of the hemangioblast to hematopoietic and endothelial cells are largely unknown. In general, much of embryonic development is coordinately r...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-02-0634
更新日期:2003-10-01 00:00:00
abstract::By using a cDNA clone of the myeloperoxidase (MPO) gene, we have studied, by Northern blot analysis, the level of MPO mRNA in eight cases of acute lymphoblastic leukemia (ALL). The blast cell populations studied were characterized by morphologic, cytochemical, immunochemical, and molecular criteria. With all the metho...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1988-09-01 00:00:00