Atypical aHUS: State of the art.


: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.


Mol Immunol


Molecular immunology


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 RJ




Has Abstract


2015-09-01 00:00:00














  • Complement activation by cholesterol crystals triggers a subsequent cytokine response.

    abstract::In the host a diverse collection of endogenous danger signals is constantly generated consisting of waste material as protein aggregates or crystalline materials that are recognized and handled by soluble pattern recognition receptors and phagocytic cells of the innate immune system. These signals may under certain ci...

    journal_title:Molecular immunology

    pub_type: 杂志文章,评审


    authors: Niyonzima N,Halvorsen B,Sporsheim B,Garred P,Aukrust P,Mollnes TE,Espevik T

    更新日期:2017-04-01 00:00:00

  • CD8low T-cell subpopulation is increased in patients with chronic hepatitis B virus infection.

    abstract::Recent studies suggest that CD8(+) T cells with down-regulated CD8 expression (CD3(+)CD8(low) T cells) represented as a distinct phenotype of CD8(+) T cells are increased and linked to disease severity in some chronically persistent infection, such as chronic HIV and parasite infection. However, the role of CD3(+)CD8(...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Ouyang L,Li X,Liang Z,Yang D,Gong F,Shen G,Weng X,Wu X

    更新日期:2013-12-01 00:00:00

  • Selection of human antibodies against cell surface-associated oligomeric anthrax protective antigen.

    abstract::The protective antigen (PA(83)) of Bacillus anthracis is the dominant antigen in natural and vaccine-induced immunity to anthrax infection. Three human single-chain variable fragments (scFvs) against cell bound PA were isolated from an antibody phage display library. Specifically, the antibodies were evaluated for the...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Steiniger SC,Altobell LJ 3rd,Zhou B,Janda KD

    更新日期:2007-04-01 00:00:00

  • BST-2/tetherin: structural biology, viral antagonism, and immunobiology of a potent host antiviral factor.

    abstract::BST-2 (also known as tetherin, CD317, or HM1.24) was first described as a potent interferon-inducible host antiviral factor nearly five years ago. Since that time, numerous reports have been published regarding the antiviral activity and immunological properties of this protein. BST-2 blocks viral replication by inhib...

    journal_title:Molecular immunology

    pub_type: 杂志文章,评审


    authors: Swiecki M,Omattage NS,Brett TJ

    更新日期:2013-06-01 00:00:00

  • Development of a humanized C1q A chain knock-in mouse: assessment of antibody independent beta-amyloid induced complement activation.

    abstract::Evidence has been accumulating for a role of inflammation in the development of Alzheimer's disease (AD), a progressive neurodegenerative disorder causing a common form of dementia in the elderly. C1q, part of the initiation component of the classical complement pathway (CCP), is associated with beta-sheet, fibrillar ...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Li M,Ager RR,Fraser DA,Tjokro NO,Tenner AJ

    更新日期:2008-06-01 00:00:00

  • 2-Methoxyestradiol protects against IgG immune complex-induced acute lung injury by blocking NF-κB and CCAAT/enhancer-binding protein β activities.

    abstract::Increasing evidences indicate that 2-Methoxyestradiol (2ME2) plays an essential role in protecting against inflammatory responses. However, its effect on IgG immune complex (IC)-induced acute lung injury (ALI) remains enigmatic. In the study, by using i.p. administration of 2ME2, we evaluated its influence on IgG IC-i...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Yan C,Shen Y,Sun Q,Yuan D,Tang H,Gao H

    更新日期:2017-05-01 00:00:00

  • Viral induction of Zac1b through TLR3- and IRF3-dependent pathways.

    abstract::Zinc finger protein regulator of apoptosis and cell cycle arrest (Zac1) is a transcription factor able to induce apoptosis or cell cycle arrest through independent pathways. In spite of the important potential functions attributed to Zac1, little is known of its physiological regulation and biological function. We dis...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Warzée B,Mesnil C,Hober D,Caloone D,Pirottin D,Garigliany MM,Desmecht D,Drion PV,Journot L,Lekeux P,Bureau F,Desmet CJ

    更新日期:2010-11-01 00:00:00

  • SOCS3 suppresses the expression of IL-4 cytokine by inhibiting the phosphorylation of c-Jun through the ERK signaling pathway in rat mast cell line RBL-2H3.

    abstract::SOCS3 is well known to negatively regulate various cytokine-mediated signaling responses, but its direct role in the expression of specific cytokines has not been clearly elucidated. To understand the role of SOCS3 in the expression of IL-4, one of the key Th2 cytokines, RBL-2H3 cells (a rat mast cell line) were engin...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Kim D,Kim SH,Cho SH,Shin K,Kim S

    更新日期:2011-02-01 00:00:00

  • The unconventional role of HLA-E: The road less traveled.

    abstract::Histocompatibility Leukocyte Antigens, or HLAs, are one of the most polymorphic molecules in humans. This high degree of polymorphism endows HLA molecules with the ability to present a vast array of peptides, an essential trait for responding to ever-evolving pathogens. Unlike classical HLA molecules (HLA-Ia), some no...

    journal_title:Molecular immunology

    pub_type: 杂志文章,评审


    authors: Grant EJ,Nguyen AT,Lobos CA,Szeto C,Chatzileontiadou DSM,Gras S

    更新日期:2020-04-01 00:00:00

  • Fugu, Takifugu ruberipes, mucus keratins act as defense molecules against fungi.

    abstract::Keratin is a cytoskeletal protein that constitutes the intermediate filament. Its distribution is restricted to epithelial tissues in mammals, but is wider in fish. An interesting feature of fish keratin is that it is abundant in the cutaneous mucus. However, the biological function of keratin in the mucus has not bee...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Shibuya K,Tsutsui S,Nakamura O

    更新日期:2019-12-01 00:00:00

  • Cellular uptake followed by class I MHC presentation of some exogenous peptides contributes to T cell stimulatory capacity.

    abstract::The T cell stimulatory activity of peptides is known to be associated with the cell surface stability and lifetime of the peptide-MHC (pepMHC) complex. In this report, soluble high-affinity T cell receptors (TCRs) that are specific for pepMHC complexes recognized by the mouse CD8+ clone 2C were used to monitor the cel...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Brophy SE,Jones LL,Holler PD,Kranz DM

    更新日期:2007-03-01 00:00:00

  • Differential regulation of accessory mitogenic signaling receptors by the T cell antigen receptor.

    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


    authors: Zhang XM,Berland R,Rosoff PM

    更新日期:1995-04-01 00:00:00

  • Calcium signaling in B cells: regulation of cytosolic Ca2+ increase and its sensor molecules, STIM1 and STIM2.

    abstract::Calcium signals are crucial for diverse cellular functions including adhesion, differentiation, proliferation, effector functions and gene expression. After engagement of the B cell receptor, the intracellular calcium ion (Ca(2+)) concentration is increased promoting the activation of various signaling cascades. While...

    journal_title:Molecular immunology

    pub_type: 杂志文章,评审


    authors: Baba Y,Matsumoto M,Kurosaki T

    更新日期:2014-12-01 00:00:00

  • Human monoclonal antibodies produced in transgenic BABkappa,lambda mice recognising idiotypic immunoglobulins of human lymphoma cells.

    abstract::Clonal idiotypic immunoglobulins of follicular lymphomas can be isolated by somatic fusion procedures. Idiotypic IgMs (Id-IgM) were isolated from two patients and used to immunise a strain of mice, deficient in mouse antibody production and engineered with yeast artificial chromosomes (YAC) containing fragments of the...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Suárez E,Yáñez R,Barrios Y,Díaz-Espada F

    更新日期:2004-07-01 00:00:00

  • Binding of bivalent ligand to cell surface IgE: can one detect ring formation?

    abstract::It is well established that aggregation of cell surface immunoglobulin is involved in signal transduction by cells of the immune system. It is less well understood what special properties of these cell surface aggregates are important in initiating the signal cascade. Several authors have proposed that cells respond t...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Posner RG,Dembo M

    更新日期:1994-12-01 00:00:00

  • Analysis of the structural diversity of monoclonal antibodies to cyclosporine.

    abstract::The immunosuppressive cyclic undecapeptide cyclosporine (Cs) represents a useful model for studying the molecular basis of antibody-antigen interactions. The three-dimensional structure of the Cs molecule is known and a large panel of monoclonal antibodies (mAbs) to Cs has been well characterized by cross-reactivity s...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Schmitter D,Poch O,Zeder G,Heinrich GF,Kocher HP,Quesniaux VF,Van Regenmortel MH

    更新日期:1990-10-01 00:00:00

  • Cloning, expression analysis and functional characterization of an interleukin-1 receptor-associated kinase 4 from Apostichopus japonicus.

    abstract::Interleukin 1 receptor-associated kinase 4 (IRAK4) is a key factor in TLR-mediated host immune function. In this study, an IRAK4 homologue molecule was identified from Apostichopus japonicus (designated as AjIRAK4) by RACE approach. The full-length cDNA of AjIRAK4 was of 2024 bp containing an open reading frame of 131...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Cui Y,Jiang L,Xing R,Wang Z,Wang Z,Shao Y,Zhang W,Zhao X,Li C

    更新日期:2018-09-01 00:00:00

  • Antigen specificity and cross-reactivity of monoclonal anti-lysozyme antibodies.

    abstract::Twenty-seven murine monoclonal anti-hen egg-white lysozyme (HEL) antibodies were tested with a panel of nine antigens; eight avian lysozymes and human lysozyme. The antibodies were arranged into 10 groups based on their antigen specificity and cross-reactivity. Antigenic determinants recognized by each group of antibo...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Harper M,Lema F,Boulot G,Poljak RJ

    更新日期:1987-02-01 00:00:00

  • Use of monoclonal antibodies to detect conformational alterations in lactate dehydrogenase isoenzyme 5 on heat denaturation and on adsorption to polystyrene plates.

    abstract::A monoclonal antibody (mAb) MF30 (IgGl) against human lactate dehydrogenase isoenzyme 5 (HLDH5) was prepared. MF30 was found to bind with high specificity to HLDH5 when the enzyme was adsorbed onto a polystyrene plate but did not recognize the isoenzyme when in solution. The isoenzymes HLDH1, HLDH2 and HLDH3 adsorbed ...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Hollander Z,Katchalski-Katzir E

    更新日期:1986-09-01 00:00:00

  • Immunochemical and related interactions with dextrans reviewed in terms of improved structural information.

    abstract::Structurally diverse dextrans from Leuconostoc mesenteroides and related bacteria have been used extensively in fundamental immunochemical studies such as induction and characterization of anti-dextran antibodies, as well as in studies of their interaction with pneumococcal antisera, normal bovine serum, concanavalin ...

    journal_title:Molecular immunology

    pub_type: 杂志文章,评审


    authors: Jeanes A

    更新日期:1986-09-01 00:00:00

  • TCR transfer induces TCR-mediated tonic inhibition of RAG genes in human T cells.

    abstract::Induction of the TCR signaling pathway terminates the expression of RAG genes, and a link between this pathway and their transcriptional control is evident from the recent demonstration of their re-expression if the TCR is subsequently lost or down-regulated. Since unstimulated T cells display a steady-state level of ...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Orlando L,Accomasso L,Circosta P,Turinetto V,Lantelme E,Porcedda P,Minieri V,Pautasso M,Willemsen RA,Cignetti A,Giachino C

    更新日期:2011-07-01 00:00:00

  • Salmonella O antigen-specific oligosaccharide-octyl conjugates activate complement via the alternative pathway at different rates depending on the structure of the O antigen.

    abstract::Artificial Salmonella serogroup B, D or Cl-specific glycolipids were prepared by covalently linking oligosaccharides corresponding to two O-antigen repeating units, obtained by phage enzyme hydrolysis of native O-antigenic polysaccharides, to octyl residues. Sheep erythrocytes coated with the artificial glycolipids we...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Grossman N,Svenson SB,Leive L,Lindberg AA

    更新日期:1990-09-01 00:00:00

  • A priori delineation of a peptide which mimics a discontinuous antigenic determinant.

    abstract::A technique was developed for identifying peptides with high affinity for a given antibody. By testing a monoclonal antibody directed against a discontinuous antigenic determinant on foot-and-mouth disease virus, peptides mimicking the determinant were identified even though the tertiary structure of the proteins comp...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Geysen HM,Rodda SJ,Mason TJ

    更新日期:1986-07-01 00:00:00

  • Role of MAIT cells in metabolic diseases.

    abstract::MAIT cells are innate-like T cells that are enriched in mucosal sites and tissues including adipose tissue and liver. They play an important role in immunity against microbial pathogens. Recently, it has been reported that MAIT cells could also be important in metabolic diseases and can be involved in setting up and m...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Toubal A,Lehuen A

    更新日期:2021-02-01 00:00:00

  • Epigenetic aspects of DC development and differentiation.

    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


    authors: Chauvistré H,Seré K

    更新日期:2020-12-01 00:00:00

  • Mass spectrometry-based relative quantification of human neutrophil peptides 1, 2, and 3 from biological samples.

    abstract::Human neutrophil peptides (HNPs) are cysteine-rich antimicrobial peptides stored in neutrophils. The similar structure of HNPs -1, -2, and -3 renders them impossible to study individually in biological samples. For the first time, we describe a method of individually identifying the HNPs -1-3 from exudative neutrophil...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Thompson L,Turko I,Murad F

    更新日期:2006-03-01 00:00:00

  • The Mycobacterium tuberculosis glycoprotein Rv1016c protein inhibits dendritic cell maturation, and impairs Th1 /Th17 responses during mycobacteria infection.

    abstract::The myobacterial factors and the associated mechanism by which Mycobacterium tuberculosis (Mtb) evades the host immune surveillance system remain widely unexplored. Here, we found that overexpressing Rv1016c, a mannosylated protein of M. tuberculosis in BCG (rBCG-Rv1016c) led to increased virulence of the recombined B...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Su H,Peng B,Zhang Z,Liu Z,Zhang Z

    更新日期:2019-05-01 00:00:00

  • Trout immunoglobulin populations differing in light chains revealed by monoclonal antibodies.

    abstract::Monoclonal antibodies (MAbs) raised against trout immunoglobulin (Ig) recognize two independent Ig populations differing in the molecular weight of L chains. The trout Ig population recognized by MAb 2H9 represented congruent to 20% of the total Ig and contained L chains congruent to 26 kDa, whereas the trout Ig popul...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Sánchez C,Domínguez J

    更新日期:1991-11-01 00:00:00

  • Effects of antibody affinity and antigen valence on molecular forms of immune complexes.

    abstract::The effect of antibody affinity on molecular forms of immune complexes was investigated by measuring antigen-antibody interactions using surface plasmon resonance (SPR), electrospray ionization time-of-flight mass spectrometry under non-denaturing conditions (MS), analytical ultracentrifugation (AUC), and transmission...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Oda M,Uchiyama S,Noda M,Nishi Y,Koga M,Mayanagi K,Robinson CV,Fukui K,Kobayashi Y,Morikawa K,Azuma T

    更新日期:2009-12-01 00:00:00

  • Influence of terminal residue on adjacent disaccharide immunogenicity.

    abstract::Aberrant O-glycosylation of cell surface mucin antigens is characteristic of epithelial cancer cells. For example, Thomsen-Friedenreich disaccharide (TFD) is a chemically well-defined carbohydrate antigen with a documented link to malignancy. There have been many attempts to improve immune response to carbohydrate ant...

    journal_title:Molecular immunology

    pub_type: 杂志文章


    authors: Irazoqui FJ,Lopez PH,Mandel U,Nores GA

    更新日期:2002-03-01 00:00:00