Abstract:
:Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.
journal_name
Mol Immunoljournal_title
Molecular immunologyauthors
Nester CM,Barbour T,de Cordoba SR,Dragon-Durey MA,Fremeaux-Bacchi V,Goodship TH,Kavanagh D,Noris M,Pickering M,Sanchez-Corral P,Skerka C,Zipfel P,Smith RJdoi
10.1016/j.molimm.2015.03.246subject
Has Abstractpub_date
2015-09-01 00:00:00pages
31-42issue
1eissn
0161-5890issn
1872-9142pii
S0161-5890(15)00350-8journal_volume
67pub_type
杂志文章,评审abstract::T cell responses are determined by the environment in which antigen is encountered. In the absence of proper costimulation, anergizing stimuli induce the activation of a specific program of gene expression. Proteins encoded by these genes impose a state of functional unresponsiveness in anergic T cells through the act...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2008.09.029
更新日期:2009-02-01 00:00:00
abstract::We have examined the regulation of the AP-1 DNA transcription complex during T cell activation in response to interleukin 1 (IL-1) and phorbol ester (TPA) treatment of the IL-1 responsive murine thymoma T cell line, EL4 6.1 C 10. IL-1 synergistically enhances the stimulatory effect of TPA on AP-1-mediated gene express...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(95)00055-j
更新日期:1995-08-01 00:00:00
abstract::The dynamics of human antigen-specific immunoglobulin (Ig) responses in early life are not well characterized. We have previously observed an inverse relationship between allergen-specific Ig concentration and allergen-Ig-binding affinity in allergen-sensitive atopic adults, suggesting a possible feedback relationship...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2004.07.006
更新日期:2005-01-01 00:00:00
abstract::The S100A12 gene belongs to the S100 family of genes, which are specific to vertebrates. It is involved in many inflammatory diseases of human and has been considered as a powerful diagnostic gene. In the present study, we identified the porcine S100A12 (pS100A12) gene, provided evidence that pS100A12 is located on ch...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2010.01.024
更新日期:2010-04-01 00:00:00
abstract::In this review we introduce the basic principles of epigenetic gene regulation and discuss them in the context of dendritic cell (DC) development and differentiation. Epigenetic mechanisms control the accessibility of chromatin for DNA binding proteins and thus they control gene expression. These mechanisms comprise c...
journal_title:Molecular immunology
pub_type: 杂志文章,评审
doi:10.1016/j.molimm.2020.10.011
更新日期:2020-12-01 00:00:00
abstract::Mangiferin is the major bioactive ingredient in the leaves of Mangifera indica L., Aqueous extract of such leaves have been traditionally used as an indigenous remedy for respiratory diseases including cough and asthma in Traditional Chinese Medicine. Mangiferin was shown to exert its anti-asthmatic effect by modulati...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2019.07.025
更新日期:2019-10-01 00:00:00
abstract::We describe the entire immunoglobulin heavy chain (IgH) locus from the reptile Anolis carolinensis. The heavy chain constant (C(H)) region includes C mu, C delta and C upsilon genes. This is the first description of a C upsilon gene in the reptilian class. Variable (V(H)), diversity (D(H)) and joining (J(H)) genes are...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2009.02.019
更新日期:2009-05-01 00:00:00
abstract::We report the construction, expression and purification of a bispecific single-chain Fv antibody fragment produced in Escherichia coli. The protein possesses a dual specificity: the single-chain FvB1 portion is directed to the Idiotype of BCL1 lymphoma cells, the single-chain Fv2C11 moiety binds to the CD3 marker on T...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(95)00089-5
更新日期:1995-12-01 00:00:00
abstract::Using the non-crossreactive mAb MRC-OX3 and MRC-OX6, two serologically distinct RT1.B-specific (I-A equivalent) alpha, beta heterodimers have previously been described by us as residing at the cell surface of LEW rat spleen cells. The two-chain elements were suggested to represent stable conformation isomers, diverged...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(89)90099-0
更新日期:1989-02-01 00:00:00
abstract::Mast cells have a clear-cut pathologic role in allergy, participating in a number of chronic inflammatory conditions, in helmintic parasitosis, and in some solid tumor reactions, but also in physiological situations, such as wound healing and innate immunity. Mast cells release a large number of proinflammatory, immun...
journal_title:Molecular immunology
pub_type: 杂志文章,评审
doi:10.1016/s0161-5890(02)00090-1
更新日期:2002-09-01 00:00:00
abstract::Human promyelocytic leukemia (HL-60) cells were induced by 1,25-dihydroxyvitamin D3 (calcitriol) to differentiate and examined using a panel of monoclonal antibodies (MoAbs) and functional assays. Although morphologically and histochemically these cells appeared to be of the monocyte-macrophage phenotype, there was a ...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(85)90180-4
更新日期:1985-05-01 00:00:00
abstract::The role of glycosylation in membrane expression and secretion of IgM and IgA was investigated in murine lymphoma and hybridoma cell lines, derived from I.29 tumor, which synthesize IgM or IgA with identical variable regions. Tunicamycin, a selective inhibitor of N-linked glycosylation, prevented the membrane expressi...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(84)90023-3
更新日期:1984-08-01 00:00:00
abstract::The five-year survival rate of melanoma worsens significantly with advancing tumor stage. We hypothesized that regulatory B cells (Breg) might have participated in the pathogenesis of melanoma. In this study, the PD-L1+ Breg cells were investigated. The expression of PD-L1 by circulating B cells was very low in health...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2020.01.008
更新日期:2020-03-01 00:00:00
abstract::In addition to the antigen receptor, resting T cells express a number of receptors that can be stimulated to generate proliferative signals. These "accessory" receptors require co-expression of the T cell receptor (TCR), suggesting that they channel their signals via secondary activation of the signal transduction fun...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(94)00160-3
更新日期:1995-04-01 00:00:00
abstract::HLA-B27 (B27) is strongly associated with the spondyloarthritides. B27 is expressed at the cell surface of antigen presenting cells (APC) both as canonical β2m-associated and non-canonical β2m-free heavy chain (FHC) forms which include B27 dimers (termed B272). B27 FHC forms arise in an endosomal compartment from recy...
journal_title:Molecular immunology
pub_type: 杂志文章,评审
doi:10.1016/j.molimm.2013.05.243
更新日期:2014-01-01 00:00:00
abstract::Amino acid sequences encoded by exon 8 of the H-2K and H-2D/L genes appear to be locus specific. The majority of H-2Kb molecules contain 10 amino acids that are derived from exon 8. In contrast, the H-2Db, -Dd and -Ld molecules contain only one amino acid which is encoded by exon 8, even though the genetic information...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(88)90165-4
更新日期:1988-05-01 00:00:00
abstract::Systemic lupus erythematosus (SLE) in patients and certain mouse models exhibits a strong sex bias. Additionally, in most patients, increased serum levels of type I interferon (IFN-α) are associated with severity of the disease. Because increased levels of B cell activating factor (BAFF) in SLE patients and mouse mode...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2012.06.013
更新日期:2013-01-01 00:00:00
abstract::The O-antigen of the gram negative bacteria Brucella is composed of an homopolymer of 4,6-dideoxy-4-formamido-alpha-D-mannopyranosyl (or perosamine). Several mAb interact specifically with only the O-antigen of certain Brucella species. Although, many studies show that this specific recognition results mainly from the...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2003.11.037
更新日期:2004-03-01 00:00:00
abstract::Comparative interactions of purified rabbit C1q with 18-2-3, a high affinity (2-3 X 10(10) M-1) anti-fluorescein (anti-F1) murine monoclonal IgM antibody (pentamer) and constitutive monomeric subunits (IgMs) were studied. Using a solid phase radioimmunoassay (SPRIA), based on immobilized polyvalent antigen, it was sho...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(88)90076-4
更新日期:1988-06-01 00:00:00
abstract::An antimicrobial polypeptide was purified from an acidified gill extract of Pacific oyster (Crassostrea gigas) by C(18) reversed-phase HPLC. The purified polypeptide had a molecular weight of 8471Da containing 74 amino acid residues. Comparison of the obtained N-terminal sequences with those of others revealed that it...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2012.07.003
更新日期:2013-01-01 00:00:00
abstract::The three-dimensional structure of the Mcg lambda-type Bence-Jones dimer crystallized in ammonium sulfate is known at 2.3-A resolution. A series of nine other human lambda-chains and two kappa-chains did not crystallize under the same conditions. After these proteins were hybridized with the Mcg light chain by the met...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/s0161-5890(85)80001-8
更新日期:1985-02-01 00:00:00
abstract::The functional importance of various cell membrane bound molecules was studied and compared in the NK cytotoxicity and CTL activity. LFA-1 and CD2 participate in both killing functions, while CD3 and CD8/CD4 as well as MHC class I molecules are involved only in CTL activity. Nevertheless CD2- and beta 2-microglobulin ...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(86)90164-1
更新日期:1986-11-01 00:00:00
abstract::The complete cDNA sequences of two bovine IgGs were obtained by RT-PCR cloning. The first-strand cDNA was prepared from an animal homozygous (A2/A2) for IgG2a; the resulting sequences of the two bovine IgGs reported here were identified as IgG2a(A2) and IgG1. These sequences, and their deduced amino acid sequences, ar...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(95)00107-7
更新日期:1996-02-01 00:00:00
abstract::Major histocompatibility complex (MHC) class I and II proteins have been in the focus of interest for immunologists, biochemists, cell biologists, and structural biologists for decades. With dozens of entries in the Protein Data Bank, their crystal structures are now sufficiently well understood, while their dynamic p...
journal_title:Molecular immunology
pub_type: 杂志文章,评审
doi:10.1016/j.molimm.2009.10.008
更新日期:2010-01-01 00:00:00
abstract::Polymorphonuclear neutrophils (PMNs) produce and release copious amounts of reactive oxygen species (ROS) which target potential bacterial invaders but also contribute to the inflammation-associated organ injuries seen in sepsis. Calprotectin is an immune regulatory protein complex made of S100A8 and S100A9 that inhib...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2012.10.002
更新日期:2013-04-01 00:00:00
abstract::Tumor-associated macrophages (TAMs) are preferentially M2 skewed and promote tumor growth, angiogenesis, invasion, and/or metastasis. In this study, we have analyzed the in vitro immunomodulatory potential of a non-toxic neem leaf glycoprotein (NLGP) in reprogramming Stage III supraglottic laryngeal tumor cell lysate ...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2014.01.015
更新日期:2014-06-01 00:00:00
abstract::The mammalian interleukin (IL)-6 family of cytokines consist of eight class-I helical cytokines and are major players in hematopoiesis, as well as in neuroendocrine and immune systems, and have pro- and anti-inflammatory properties. We have identified a novel fish CNTF-like molecule, for the first time, as well as the...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2009.04.003
更新日期:2009-07-01 00:00:00
abstract::TRAF6 is a key player at the cross-roads of development and immunity. The analysis of its in vivo molecular function is a great challenge since severe developmental defects and early lethality caused by Traf6 deficiency in knock-out mice interfere with analyses of the immune response. In this study we have used a new ...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/j.molimm.2010.08.011
更新日期:2010-11-01 00:00:00
abstract::Membrane immunoglobulins are integral proteins on B cell surfaces that bind foreign antigens and are critically involved in the regulation of the immune response. Based upon the model of serum IgG, it has been assumed that membrane immunoglobulins are essentially four chain disulfide-linked structures of the form H2L2...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/0161-5890(93)90451-g
更新日期:1993-12-01 00:00:00
abstract::The regulatory role of MEF2 (myocyte enhancer binding factor 2) proteins in nonmuscle tissues has not been well characterized. We examined the expression of MEF2 family members, namely, MEF2A, -B, -C, and -D, in the differentiation of HL60 promyeloid cells and observed the remarkable increase in the expressions of MEF...
journal_title:Molecular immunology
pub_type: 杂志文章
doi:10.1016/s0161-5890(99)00140-6
更新日期:1999-12-01 00:00:00