- Defects in Glanzmann thrombasthenia and LAD-III (LAD-1/v) syndrome: the role of integrin beta 1 and beta 3 in platelet adhesion to collagen
[作者:van de Vijver, E; De Cuyper, IM; Gerrits, AJ; Verhoeven, AJ; Seeger, K; Gutierrez, L; van den Berg, TK; Kuijpers, TW,期刊:Blood, 页码:583-586 , 文章类型: Article,,卷期:2012年119-2]
- Patients with Glanzmann thrombasthenia or Leukocyte Adhesion Deficiency-III syndrome (LAD-III or LAD-1/variant) present with increased bleeding tendency because of the lack or dysfunction of the fibrinogen receptor GPIIb...
- Deletion of HIF-2 alpha in the enterocytes decreases the severity of tissue iron loading in hepcidin knockout mice
[作者:Mastrogiannaki, M; Matak, P; Delga, S; Deschemin, JC; Vaulont, S; Peyssonnaux, C,期刊:Blood, 页码:587-590 , 文章类型: Article,,卷期:2012年119-2]
- Hereditary hemochromatosis (HH) is a highly prevalent genetic disorder characterized by excessive parenchymal iron accumulation leading to liver cirrhosis, diabetes, and in some cases hepatocellular carcinoma. HH is caus...
- Anovel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome
[作者:Francis, NJ; McNicholas, B; Awan, A; Waldron, M; Reddan, D; Sadlier, D; Kavanagh, D; Strain, L; Marchbank, KJ; Harris, CL; Goodship, THJ,期刊:Blood, 页码:591-601 , 文章类型: Article,,卷期:2012年119-2]
- Genomic disorders affecting the genes encoding factor H (fH) and the 5 factor H related proteins have been described in association with atypical hemolytic uremic syndrome. These include deletions of CFHR3, CFHR1, and CF...
- Engineered factor IX variants bypass FVIII and correct hemophilia A phenotype in mice
[作者:Milanov, P; Ivanciu, L; Abriss, D; Quade-Lyssy, P; Miesbach, W; Alesci, S; Tonn, T; Grez, M; Seifried, E; Schuttrumpf, J,期刊:Blood, 页码:602-611 , 文章类型: Article,,卷期:2012年119-2]
- The complex of the serine protease factor IX (FIX) and its cofactor, factor VIII (FVIII), is crucial for propagation of the intrinsic coagulation cascade. Absence of either factor leads to hemophilia, a disabling disorde...
- Population pharmacokinetics of recombinant factor VIII: the relationships of pharmacokinetics to age and body weight
[作者:Bjorkman, S; Oh, M; Spotts, G; Schroth, P; Fritsch, S; Ewenstein, BM; Casey, K; Fischer, K; Blanchette, VS; Collins, PW,期刊:Blood, 页码:612-618 , 文章类型: Article,,卷期:2012年119-2]
- Comparison of the pharmacokinetics (PK) of a coagulation factor between groups of patients can be biased by differences in study protocols, in particular between blood sampling schedules. This could affect clinical dose ...
- Blocking IL-21 signaling ameliorates xenogeneic GVHD induced by human lymphocytes
[作者:Hippen, KL; Bucher, C; Schirm, DK; Bearl, AM; Brender, T; Mink, KA; Waggie, KS; de Latour, RP; Janin, A; Curtsinger, JM; Dillon, SR; Miller, JS; Socie, G; Blazar, BR,期刊:Blood, 页码:619-628 , 文章类型: Article,,卷期:2012年119-2]
- In rodent graft-versus-host disease (GVHD) models, anti-IL-21 neutralizing mAb treatment ameliorates lethality and is associated with decreases in Th1 cytokine production and gastrointestinal tract injury. GVHD preventio...
- Relaxin increases human endothelial progenitor cell NO and migration and vasculogenesis in mice
[作者:Segal, MS; Sautina, L; Li, SY; Diao, YP; Agoulnik, AI; Kielczewski, J; McGuane, JT; Grant, MB; Conrad, KP,期刊:Blood, 页码:629-636 , 文章类型: Article,,卷期:2012年119-2]
- The ovarian peptide hormone, relaxin, circulates during pregnancy, contributing to profound maternal vasodilation through endothelial and nitric oxide (NO)-dependent mechanisms. Circulating numbers of bone marrow-derived...
- Identification of VLDLR as a novel endothelial cell receptor for fibrin that modulates fibrin-dependent transendothelial migration of leukocytes
[作者:Yakovlev, S; Mikhailenko, I; Cao, CZ; Zhang, L; Strickland, DK; Medved, L,期刊:Blood, 页码:637-644 , 文章类型: Article,,卷期:2012年119-2]
- While testing the effect of the (beta 15-66)(2) fragment, which mimics a pair of fibrin beta N-domains, on the morphology of endothelial cells, we found that this fragment induces redistribution of vascular endothelial-c...
- Plasmodium falciparum STEVOR proteins impact erythrocyte mechanical properties
[作者:Sanyal, S; Egee, S; Bouyer, G; Perrot, S; Safeukui, I; Bischoff, E; Buffet, P; Deitsch, KW; Mercereau-Puijalon, O; David, PH; Templeton, TJ; Lavazec, C,期刊:Blood, 页码:E1-E8 , 文章类型: Editorial Material,,卷期:2012年119-2]
- Infection of erythrocytes with the human malaria parasite, Plasmodium falciparum, results in dramatic changes to the host cell structure and morphology. The predicted functional localization of the STEVOR proteins at the...
- NOTCH1 mutations in CLL associated with trisomy 12
[作者:Balatti, V; Bottoni, A; Palamarchuk, A; Alder, H; Rassenti, LZ; Kipps, TJ; Pekarsky, Y; Croce, CM,期刊:Blood, 页码:329-331 , 文章类型: Article,,卷期:2012年119-2]
- Two recent studies reported whole-genome sequencing of chronic lymphocytic leukemia (CLL) samples and found repeated mutations in the XPO1 and NOTCH1 genes. XPO1 was found mutated in 2.4% of cases, while NOTCH1 was found...
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