Disorders of apoptosis: mechanisms for autoimmunity in primary immunodeficiency diseases.


:A number of primary immunodeficiency diseases represent a paradox of immunodeficiency and autoimmunity. In this minireview, we present basic concepts of apoptosis and disorder of apoptosis as one of the mechanisms to explain such a paradox between immunodeficiency and autoimmunity, which is exemplified by autoimmune lymphoproliferative syndrome (ALPS).


J Clin Immunol


Oliveira JB,Gupta S




Has Abstract


2008-05-01 00:00:00








28 Suppl 1


  • Insights into Mutation Effect in Three Poikiloderma with Neutropenia Patients by Transcript Analysis and Disease Evolution of Reported Patients with the Same Pathogenic Variants.

    abstract:PURPOSE:Poikiloderma with neutropenia (PN) is a genodermatosis currently described in 77 patients, all presenting with early-onset poikiloderma, neutropenia, and several additional signs. Biallelic loss-of-function mutations in USB1 gene are detected in all molecularly tested patients but genotype-phenotype correlation...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Colombo EA,Elcioglu NH,Graziano C,Farinelli P,Di Fede E,Neri I,Facchini E,Greco M,Gervasini C,Larizza L

    更新日期:2018-05-01 00:00:00

  • Genetic polymorphisms in the CD40 ligand gene and Kawasaki disease.

    abstract:BACKGROUND:Although some previous studies have reported that genetic and immunological factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiological factors of this enigmatical pediatric disease are still poorly understood. PURPOSE:This study aims to investigate whether polymorphisms of th...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Huang FY,Chang TY,Chen MR,Chiu NC,Chi H,Lee HC,Lin SP,Chen CK,Chan HW,Chen WF,Liu HF,Chu CC,Lin M,Lee YJ

    更新日期:2008-09-01 00:00:00

  • Immunogenicity of human immunodeficiency virus (HIV) reverse transcriptase: detection of high levels of antibodies to HIV reverse transcriptase in sera of homosexual men.

    abstract::Immunoglobulin isolated from sera of homosexual men infected with human immunodeficiency virus (HIV) inhibited the reverse transcriptase (RT) activity of HIV. The inhibitory activity was specifically directed against HIV RT, and not against other mammalian retrovirus RT, including human T-lymphotropic virus type I. Th...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Chatterjee R,Rinaldo CR Jr,Gupta P

    更新日期:1987-05-01 00:00:00

  • Post-transplantation B cell function in different molecular types of SCID.

    abstract:PURPOSE:Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T, B and sometimes NK cell function. Non-ablative HLA-identical or rigorously T cell-depleted haploidentical parental bone marrow transplantation (BMT) results in thymus-dependent genetically...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Buckley RH,Win CM,Moser BK,Parrott RE,Sajaroff E,Sarzotti-Kelsoe M

    更新日期:2013-01-01 00:00:00

  • Antiidiotypic response against murine monoclonal antibodies reactive with tumor-associated antigen TAG-72.

    abstract::The immune response of 42 gastrointestinal and ovarian cancer patients at 1 month after exposure to murine monoclonal antibodies (B72.3 and CC49) reactive with the tumor-associated antigen TAG-72 was studied. The incidence of human anti-mouse antibody response was 89% to B72.3 and 70% to CC49. To evaluate the antiidio...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Blanco I,Kawatsu R,Harrison K,Leichner P,Augustine S,Baranowska-Kortylewicz J,Tempero M,Colcher D

    更新日期:1997-01-01 00:00:00

  • Rapid subcutaneous IgG replacement therapy is effective and safe in children and adults with primary immunodeficiencies--a prospective, multi-national study.

    abstract::Sixty patients (16 children, 44 adults) participated in the study aiming at evaluating: (i) IgG levels when switching patients from intravenous IgG (IVIG) infusions in hospital to subcutaneous (SCIG) self-infusions at home using the same cumulative monthly dose, (ii) protections against infections, and (iii) safety of...

    journal_title:Journal of clinical immunology

    pub_type: 临床试验,杂志文章,多中心研究


    authors: Gardulf A,Nicolay U,Asensio O,Bernatowska E,Böck A,Carvalho BC,Granert C,Haag S,Hernández D,Kiessling P,Kus J,Pons J,Niehues T,Schmidt S,Schulze I,Borte M

    更新日期:2006-03-01 00:00:00

  • The transfer of antigen-specific humoral immunity from marrow donors to marrow recipients.

    abstract::This study shows that specific humoral immunity could be transferred from marrow donors to marrow recipients. Peripheral blood lymphocytes from long-term human marrow recipients produced IgG anti-tetanus toxoid antibody after in vitro tetanus toxoid stimulation. Antitetanus toxoid antibody biosynthesis was induced usi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Lum LG,Seigneuret MC,Storb R

    更新日期:1986-09-01 00:00:00

  • Matrix metalloproteinase-9 and autoimmune diseases.

    abstract::Matrix metalloproteinases (also named matrixin or MMPs) are a major group of enzymes that regulate cell-matrix composition by using zinc for their proteolytic activities. They are essential for various normal biological processes such as embryonic development, morphogenesis, reproduction tissue resorption, and remodel...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审


    authors: Ram M,Sherer Y,Shoenfeld Y

    更新日期:2006-07-01 00:00:00

  • Regulation of ERK2 dephosphorylation in G1-stimulated rat T lymphoblasts.

    abstract::Rat T lymphoblasts arrested in the G1 phase of the cell cycle by interleukin-2 (IL-2) deprivation can be forced to proceed to the S phase when they are stimulated with IL-2 or the phorbol ester phorbol 12,13-dibutyrate (PDBu). When PDBu is used as a stimulus, extracellular regulated kinase 2 (ERK2) is activated by thr...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Lisbona C,Alemany S,Fernández-Renart M

    更新日期:1997-11-01 00:00:00

  • Transduction of primary lymphocytes with Epstein-Barr virus (EBV) latent membrane protein-specific T-cell receptor induces lysis of virus-infected cells: A novel strategy for the treatment of Hodgkin's disease and nasopharyngeal carcinoma.

    abstract::Adoptive immunotherapy with in vitro expanded cytotoxic T lymphocytes specific for Epstein-Barr virus (EBV) can successfully treat post-transplant lymphoproliferative disease (PTLD). However, extension of a similar strategy to Hodgkin's disease (HD) and nasopharyngeal carcinoma (NPC) is limited by the poor immunogenic...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Jurgens LA,Khanna R,Weber J,Orentas RJ

    更新日期:2006-01-01 00:00:00

  • A Defective Oxidative Burst and Impaired Antigen Presentation are Hallmarks of Human Visceral Leishmaniasis.

    abstract:PURPOSE:Survival of the Leishmania parasite within monocytes hinges on its ability to effectively nullify their microbicidal effector mechanisms. Accordingly, this study aimed to delineate this biological niche in patients with visceral leishmaniasis (VL). METHODS:In monocytes, the redox status, antigen presenting cap...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Roy S,Mukhopadhyay D,Mukherjee S,Ghosh S,Kumar S,Sarkar K,Pal D,Bhowmik P,Mandal K,Modak D,Guha SK,Pramanik N,Goswami RP,Saha B,Chatterjee M

    更新日期:2015-01-01 00:00:00

  • Characterizing the immune system after long-term undetectable viral load in HIV-1-infected children.

    abstract::Thirty two HIV-infected children, on highly active antiretroviral therapy (HAART) and > 500 CD4+ T cells/mm3, were rated according to the time-course of viral load (VL) during the whole follow-up period (> 18 months) in a longitudinal retrospective study. (a) uVL group: 15 children with VL below 400 copies/mL; (b) dVL...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Resino S,Galán I,Bellón JM,Navarro ML,León JA,Muñoz-Fernandez MA

    更新日期:2003-07-01 00:00:00

  • N-acetylcysteine administration is associated with reduced activation of NF-kB and preserves lung dendritic cells function in a zymosan-induced generalized inflammation model.

    abstract:PURPOSE:In severe sepsis, functional impairment and decreased numbers of dendritic cells (DCs) are essential reasons for immune function paralysis, secondary organ infection, and organ failure. We investigated the effects of N-acetylcysteine (NAC) administration on protecting lung DCs function in a zymosan-induced gene...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Wang HW,Yang W,Lu JY,Li F,Sun JZ,Zhang W,Guo NN,Gao L,Kang JR

    更新日期:2013-04-01 00:00:00

  • Differential expression of perforin in cytotoxic lymphocyte in HIV/AIDS patients of China.

    abstract::Cytotoxic lymphocytes are critical in the control of HIV replication, it has been shown that perforin is the key effector of killing machinery for CTLs and NK cells, so we investigated the circulating levels of perforin in CD8+ T cells and NK cells by flow cytometry intracellular stain in Chinese HIV infected individu...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Qi W,Yongjun J,Yanan W,Zining Z,Xiaoxu H,Jing L,Hong S

    更新日期:2006-07-01 00:00:00

  • Probabilistic regulation of IL-4 production.

    abstract::Among a population of uniformly differentiated TH(2) cells, only a portion express IL-4 upon stimulation and those that do often express the product of only a single allele. We review the evidence for the basis of IL-4 monoallelism and argue that it depends upon probabilistic expression of the Il4 gene. Further, we ar...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审


    authors: Guo L,Hu-Li J,Paul WE

    更新日期:2005-11-01 00:00:00

  • Molecular, Immunological, and Clinical Features of 16 Iranian Patients with Mendelian Susceptibility to Mycobacterial Disease.

    abstract:PURPOSE:Mendelian susceptibility to mycobacterial disease (MSMD) is a rare primary immunodeficiency, triggered by non-tuberculous mycobacteria or Bacillus Calmette-Guérin (BCG) vaccines and characterized by severe diseases. All known genetic etiologies are inborn errors of IFN-γ-mediated immunity. Here, we report the m...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Sarrafzadeh SA,Nourizadeh M,Mahloojirad M,Fazlollahi MR,Shokouhi Shoormasti R,Badalzadeh M,Deswarte C,Casanova JL,Pourpak Z,Bustamante J,Moin M

    更新日期:2019-04-01 00:00:00

  • Modulation of the proteome of peripheral blood mononuclear cells from HIV-1-infected patients by drugs of abuse.

    abstract:INTRODUCTION:We used proteomic analyses to assess how drug abuse modulates immunologic responses to infections with the human immunodeficiency virus type 1 (HIV-1). METHODS:Two-dimensional difference gel electrophoresis was utilized to determine changes in the proteome of peripheral blood mononuclear cells (PBMC) isol...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Reynolds JL,Mahajan SD,Aalinkeel R,Nair B,Sykes DE,Agosto-Mujica A,Hsiao CB,Schwartz SA

    更新日期:2009-09-01 00:00:00

  • Primary immunodeficiency disorders in the Republic of Ireland: first report of the national registry in children and adults.

    abstract::Data collection for the national registry for patients with primary immunodeficiency disorders in the Republic of Ireland commenced in 1996. One hundred and fifteen cases of primary immunodeficiency diseases were registered between December 1996 and February 2003. The most frequent primary immunodeficiency disorders w...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Abuzakouk M,Feighery C

    更新日期:2005-01-01 00:00:00

  • Clinical and molecular characteristics of 35 Chinese children with Wiskott-Aldrich syndrome.

    abstract:BACKGROUND:Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease, with an incidence of 4/1,000,000 live male births. In China, an estimated number of 35 babies with WAS are born each year, but likely many remain undiagnosed. OBJECTIVES:The objectives of study were to review the clinical and molecul...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Lee PP,Chen TX,Jiang LP,Chen J,Chan KW,Lee TL,Ho MH,Nong SH,Yang Y,Fang YJ,Li Q,Wang XC,Yang XQ,Lau YL

    更新日期:2009-07-01 00:00:00

  • Inhibition of the release of soluble tumor necrosis factor receptors in experimental endotoxemia by an anti-tumor necrosis factor-alpha antibody.

    abstract::The role of tumor necrosis factor-alpha in the shedding of soluble tumor necrosis factor receptors in endotoxemia was investigated. The appearance of the soluble tumor necrosis factor receptors was assessed in four healthy volunteers following an intravenous injection of tumor necrosis factor-alpha and in eight chimpa...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Jansen J,van der Poll T,Levi M,ten Cate H,Gallati H,ten Cate JW,van Deventer SJ

    更新日期:1995-01-01 00:00:00

  • Severe combined immunodeficiency (SCID) in Canadian children: a national surveillance study.

    abstract:PURPOSE:Severe Combined Immune Deficiency (SCID) is universally fatal unless treated with hematopoietic stem cell transplantation (HSCT). Following the identification of disseminated Bacille Calmette-Guérin (BCG) infections in Canadian First Nations, Métis and Inuit (FNMI) children with unrecognized primary immune defi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Rozmus J,Junker A,Thibodeau ML,Grenier D,Turvey SE,Yacoub W,Embree J,Haddad E,Langley JM,Ramsingh RM,Singh VA,Long R,Schultz KR

    更新日期:2013-11-01 00:00:00

  • Probing the human B-cell repertoire: isolation of Epstein-Barr virus (EBV)-transformed human B lymphocytes making antibodies with a common idiotope that have different antigen-binding specificities.

    abstract::Human peripheral B lymphocytes were transformed with Epstein-Barr virus and probed with an antiidiotypic antibody made against a human monoclonal autoantibody. Six cell lines were isolated that produced antibodies bearing a common idiotope. Despite the presence of this common idiotope, the antibodies showed antigen-bi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Uchigata Y,Prabhakar BS,Notkins AL

    更新日期:1988-11-01 00:00:00

  • i.v.IG for autoimmune, fibrosis, and malignant conditions: our experience with 200 patients.

    abstract::Intravenous immunoglobin (i.v.IG) is generated from the plasma of more than 10,000 healthy subjects. Originally i.v.IG was employed to supplement the missing Igs in subjects with immune-deficiencies. Later on it was given successfully to patients with autoimmune conditions, in some of which the benefits were confirmed...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审


    authors: Shoenfeld Y,Krause I

    更新日期:2004-03-01 00:00:00

  • Primary immunodeficiency disorders in Iran: update and new insights from the third report of the national registry.

    abstract:BACKGROUND:Primary immunodeficiency disorders (PID) are a group of heterogeneous disorders mainly characterized by severe and recurrent infections and increased susceptibility to malignancies, lymphoproliferative and autoimmune conditions. National registries of PID disorders provide epidemiological data and increase t...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,多中心研究


    authors: Aghamohammadi A,Mohammadinejad P,Abolhassani H,Mirminachi B,Movahedi M,Gharagozlou M,Parvaneh N,Zeiaee V,Mirsaeed-Ghazi B,Chavoushzadeh Z,Mahdaviani A,Mansouri M,Yousefzadegan S,Sharifi B,Zandieh F,Hedayat E,Nadjafi A,S

    更新日期:2014-05-01 00:00:00

  • Long-term follow-up of health in blood donors with primary selective IgA deficiency.

    abstract::A 20-year health follow-up study of 159 initially healthy blood donors with a severe deficiency of serum IgA ( < 0.05 x 10(-3) g/L) and of 45 donors with decreased serum IgA (0.05 x 10(-3)-0.8 g/L) was carried out. The findings indicate that persons with a severe deficiency of and decreased serum IgA who are healthy a...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Koskinen S

    更新日期:1996-05-01 00:00:00

  • APDS2 and SHORT Syndrome in a Teenager with PIK3R1 Pathogenic Variant.

    abstract::Activated PI3K δ syndrome (APDS) is a primary immunodeficiency caused by heterogeneous germline gain-of-function mutations which ultimately lead to the hyperactivation of the phosphoinositide-3-kinase δ (PI3K δ). PI3K δ exists as a heterodimer composed of a catalytic and a regulatory subunit. APDS type 2 is caused by ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Ramirez L,Tamayo W,Ale H

    更新日期:2020-10-01 00:00:00

  • Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity.

    abstract::Live-attenuated vaccines (LAVs) can protect humans against 12 viral and three bacterial diseases. By definition, any clinical infection caused by a LAV that is sufficiently severe to require medical intervention attests to an inherited or acquired immunodeficiency that must be diagnosed or identified. Self-healing inf...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审


    authors: Pöyhönen L,Bustamante J,Casanova JL,Jouanguy E,Zhang Q

    更新日期:2019-05-01 00:00:00

  • BAFF, a new target for intravenous immunoglobulin in autoimmunity and cancer.

    abstract::Intravenous immunoglobulin (IVIg) has been used to treat autoimmune diseases and lymphoid malignancies with some therapeutic effect. In both these pathological conditions, there is an overproduction of BAFF (for "B-cell-activating factor of the TNF family"), and APRIL (for "a proliferation-inducing ligand"). The prese...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Le Pottier L,Bendaoud B,Dueymes M,Daridon C,Youinou P,Shoenfeld Y,Pers JO

    更新日期:2007-05-01 00:00:00

  • Characterizing the pregnancy immune phenotype: results of the viral immunity and pregnancy (VIP) study.

    abstract:PURPOSE:The increased risk of morbidity and mortality from certain microbial infections and the demonstrated improvements in the clinical course of some autoimmune diseases support the existence of pregnancy-related alterations in immune status. Elucidating the changes in innate and adaptive immunity during gestation m...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Kraus TA,Engel SM,Sperling RS,Kellerman L,Lo Y,Wallenstein S,Escribese MM,Garrido JL,Singh T,Loubeau M,Moran TM

    更新日期:2012-04-01 00:00:00

  • 30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators.

    abstract:PURPOSE:To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable descriptive data, but lack risk stratification to guide physicians in...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章


    authors: Baloh C,Reddy A,Henson M,Prince K,Buckley R,Lugar P

    更新日期:2019-10-01 00:00:00