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  1. AU="Ben Mustapha, Imen"
  2. AU="Kong, Xueqian"
  3. AU="Phan, Ngoc Minh Hien"
  4. AU="Khan, Tazeen"
  5. AU="Adzhubei, Ivan A"
  6. AU="Alicia Reyes-Arellano"
  7. AU="L. Marcus Wilhelmsson"
  8. AU=Filocamo Giovanni
  9. AU="Andrea Terán-Valdez"
  10. AU=Cleverley Joanne AU=Cleverley Joanne
  11. AU="Feng, Shiguang"
  12. AU="De Falco, Antonio"
  13. AU="Plenter, R J"
  14. AU="Malarz, Janusz"

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  1. Artikel ; Online: Novel insights into FAS defects underlying autoimmune lymphoproliferative syndrome revealed by studies in consanguineous patients.

    Ben-Mustapha, Imen / Agrebi, Nourhen / Barbouche, Mohamed-Ridha

    Journal of leukocyte biology

    2017  Band 103, Heft 3, Seite(n) 501–508

    Abstract: Autoimmune lymphoproliferative syndrome (ALPS) is a primary immunodeficiency disease due to impaired Fas-Fas ligand apoptotic pathway. It is characterized by chronic nonmalignant, noninfectious lymphadenopathy and/or splenomegaly associated with ... ...

    Abstract Autoimmune lymphoproliferative syndrome (ALPS) is a primary immunodeficiency disease due to impaired Fas-Fas ligand apoptotic pathway. It is characterized by chronic nonmalignant, noninfectious lymphadenopathy and/or splenomegaly associated with autoimmune manifestations primarily directed against blood cells. Herein, we review the heterogeneous ALPS molecular bases and discuss recent findings revealed by the study of consanguineous patients. Indeed, this peculiar genetic background favored the identification of a novel form of AR ALPS-FAS associated with normal or residual protein expression, expanding the spectrum of ALPS types. In addition, rare mutational mechanisms underlying the splicing defects of FAS exon 6 have been identified in AR ALPS-FAS with lack of protein expression. These findings will help decipher critical regions required for the tight regulation of FAS exon 6 splicing. We also discuss the genotype-phenotype correlation and disease severity in AR ALPS-FAS. Altogether, the study of ALPS molecular bases in endogamous populations helps to better classify the disease subgroups and to unravel the Fas pathway functioning.
    Mesh-Begriff(e) Autoimmune Lymphoproliferative Syndrome/genetics ; Autoimmune Lymphoproliferative Syndrome/immunology ; Consanguinity ; Humans ; Mutation ; Severity of Illness Index ; fas Receptor/deficiency ; fas Receptor/genetics ; fas Receptor/immunology
    Chemische Substanzen FAS protein, human ; fas Receptor
    Sprache Englisch
    Erscheinungsdatum 2017-12-27
    Erscheinungsland United States
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 605722-6
    ISSN 1938-3673 ; 0741-5400
    ISSN (online) 1938-3673
    ISSN 0741-5400
    DOI 10.1002/JLB.5MR0817-332R
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  2. Artikel ; Online: In silico comparative study of SARS-CoV-2 proteins and antigenic proteins in BCG, OPV, MMR and other vaccines: evidence of a possible putative protective effect.

    Haddad-Boubaker, Sondes / Othman, Houcemeddine / Touati, Rabeb / Ayouni, Kaouther / Lakhal, Marwa / Ben Mustapha, Imen / Ghedira, Kais / Kharrat, Maher / Triki, Henda

    BMC bioinformatics

    2021  Band 22, Heft 1, Seite(n) 163

    Abstract: Background: Coronavirus Disease 2019 (COVID-19) is a viral pandemic disease that may induce severe pneumonia in humans. In this paper, we investigated the putative implication of 12 vaccines, including BCG, OPV and MMR in the protection against COVID-19. ...

    Abstract Background: Coronavirus Disease 2019 (COVID-19) is a viral pandemic disease that may induce severe pneumonia in humans. In this paper, we investigated the putative implication of 12 vaccines, including BCG, OPV and MMR in the protection against COVID-19. Sequences of the main antigenic proteins in the investigated vaccines and SARS-CoV-2 proteins were compared to identify similar patterns. The immunogenic effect of identified segments was, then, assessed using a combination of structural and antigenicity prediction tools.
    Results: A total of 14 highly similar segments were identified in the investigated vaccines. Structural and antigenicity prediction analysis showed that, among the identified patterns, three segments in Hepatitis B, Tetanus, and Measles proteins presented antigenic properties that can induce putative protective effect against COVID-19.
    Conclusions: Our results suggest a possible protective effect of HBV, Tetanus and Measles vaccines against COVID-19, which may explain the variation of the disease severity among regions.
    Mesh-Begriff(e) Antigens, Viral/immunology ; BCG Vaccine ; COVID-19 ; COVID-19 Vaccines ; Computer Simulation ; Cross Protection ; Humans ; Protein Conformation ; SARS-CoV-2/chemistry ; Viral Proteins/immunology ; Viral Vaccines/immunology
    Chemische Substanzen Antigens, Viral ; BCG Vaccine ; COVID-19 Vaccines ; Viral Proteins ; Viral Vaccines
    Sprache Englisch
    Erscheinungsdatum 2021-03-26
    Erscheinungsland England
    Dokumenttyp Journal Article
    ZDB-ID 2041484-5
    ISSN 1471-2105 ; 1471-2105
    ISSN (online) 1471-2105
    ISSN 1471-2105
    DOI 10.1186/s12859-021-04045-3
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  3. Artikel ; Online: Case Report:

    Rais, Afef / Mekki, Najla / Fedhila, Faten / Alosaimi, Mohammed Faraj / Ben Khaled, Monia / Zameli, Amal / Agrebi, Nourhen / Sellami, Maryam Kallel / Geha, Raif / Ben-Mustapha, Imen / Barbouche, Mohamed-Ridha

    Frontiers in immunology

    2021  Band 12, Seite(n) 692107

    Abstract: ALPS and IPEX are two well-characterized inborn errors of immunity with immune dysregulation, considered as two master models of monogenic auto-immune diseases. Thus, with autoimmunity as their primary clinical manifestation, these two entities may show ... ...

    Abstract ALPS and IPEX are two well-characterized inborn errors of immunity with immune dysregulation, considered as two master models of monogenic auto-immune diseases. Thus, with autoimmunity as their primary clinical manifestation, these two entities may show clinical overlap. Traditionally, immunological biomarkers are used to establish an accurate differential diagnosis. Herein, we describe a patient who presented with clinical features and biomarkers fulfilling the diagnostic criteria of ALPS. Severe apoptotic defect was also shown in the patient's cell lines and PHA-activated peripheral blood lymphocytes. Sanger sequencing of the
    Mesh-Begriff(e) Autoimmune Lymphoproliferative Syndrome/diagnosis ; Autoimmune Lymphoproliferative Syndrome/genetics ; Autoimmune Lymphoproliferative Syndrome/immunology ; Child ; Forkhead Transcription Factors/genetics ; Humans ; Male ; Mutation
    Chemische Substanzen FOXP3 protein, human ; Forkhead Transcription Factors
    Sprache Englisch
    Erscheinungsdatum 2021-08-31
    Erscheinungsland Switzerland
    Dokumenttyp Case Reports ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2021.692107
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  4. Artikel ; Online: Profound differences in IgE and IgG recognition of micro-arrayed allergens in hyper-IgE syndromes.

    Garib, Victoria / Ben-Ali, Meriem / Kundi, Michael / Curin, Mirela / Yaakoubi, Roukaya / Ben-Mustapha, Imen / Mekki, Najla / Froeschl, Renate / Perkmann, Thomas / Valenta, Rudolf / Barbouche, Mohamed-Ridha

    Allergy

    2021  Band 77, Heft 6, Seite(n) 1761–1771

    Abstract: Background: The specificities of IgE and IgG for allergen molecules in patients with inborn errors of immunity (IEI) have not been investigated in detail.: Objective: To study IgE and IgG antibody specificities in patients with defined hyper-IgE ... ...

    Abstract Background: The specificities of IgE and IgG for allergen molecules in patients with inborn errors of immunity (IEI) have not been investigated in detail.
    Objective: To study IgE and IgG antibody specificities in patients with defined hyper-IgE syndromes (HIES) using a comprehensive panel of allergen molecules.
    Methods: We used chips containing micro-arrayed allergen molecules to analyze allergen-specific IgE and IgG levels in sera from two groups of HIES patients: Autosomal recessive mutations in phosphoglucomutase-3 (PGM3); Autosomal dominant negative mutations of STAT3 (STAT3); and age-matched subjects with allergic sensitizations. Assays with rat basophil leukemia cells transfected with human FcεRI were performed to study the biological relevance of IgE sensitizations.
    Results: Median total IgE levels were significantly lower in the sensitized control group (212.9 kU/L) as compared to PGM3 (5042 kU/L) and STAT3 patients (2561 kU/L). However, PGM3 patients had significantly higher allergen-specific IgE levels and were sensitized to a larger number of allergen molecules as compared to STAT3 patients. Biological relevance of IgE sensitization was confirmed for PGM3 patients by basophil activation testing. PGM3 patients showed significantly lower cumulative allergen-specific IgG responses in particular to milk and egg allergens as compared to STAT3 patients and sensitized controls whereas total IgG levels were comparable to STAT3 patients and significantly higher than in controls.
    Conclusion: The analysis with multiple micro-arrayed allergen molecules reveals profound differences of allergen-specific IgE and IgG recognition in PGM3 and STAT3 patients which may be useful for classification of IEI and clinical characterization of patients.
    Mesh-Begriff(e) Allergens ; Humans ; Immunoglobulin E ; Immunoglobulin G/genetics ; Job Syndrome/diagnosis ; Job Syndrome/genetics ; Mutation
    Chemische Substanzen Allergens ; Immunoglobulin G ; Immunoglobulin E (37341-29-0)
    Sprache Englisch
    Erscheinungsdatum 2021-11-02
    Erscheinungsland Denmark
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 391933-x
    ISSN 1398-9995 ; 0105-4538
    ISSN (online) 1398-9995
    ISSN 0105-4538
    DOI 10.1111/all.15143
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  5. Artikel: Lessons from Genetic Studies of Primary Immunodeficiencies in a Highly Consanguineous Population.

    Barbouche, Mohamed-Ridha / Mekki, Najla / Ben-Ali, Meriem / Ben-Mustapha, Imen

    Frontiers in immunology

    2017  Band 8, Seite(n) 737

    Abstract: During the last decades, the study of primary immunodeficiencies (PIDs) has contributed tremendously to unravel novel pathways involved in a variety of immune responses. Many of these PIDs have an autosomal recessive (AR) mode of inheritance. Thus, the ... ...

    Abstract During the last decades, the study of primary immunodeficiencies (PIDs) has contributed tremendously to unravel novel pathways involved in a variety of immune responses. Many of these PIDs have an autosomal recessive (AR) mode of inheritance. Thus, the investigation of the molecular basis of PIDs is particularly relevant in consanguineous populations from Middle East and North Africa (MENA). Although significant efforts have been made in recent years to develop genetic testing across the MENA region, few comprehensive studies reporting molecular basis of PIDs in these settings are available. Herein, we review genetic characteristics of PIDs identified in 168 patients from an inbred Tunisian population. A spectrum of 25 genes involved was analyzed. We show that AR forms compared to X-linked or autosomal dominant forms are clearly the most frequent. Furthermore, the study of informative consanguineous families did allow the identification of a novel hyper-IgE syndrome linked to phosphoglucomutase 3 mutations. We did also report a novel form of autoimmune lymphoproliferative syndrome caused by homozygous FAS mutations with normal or residual protein expression as well as a novel AR transcription factor 3 deficiency. Finally, we identified several founder effects for specific AR mutations. This did facilitate the implementation of preventive approaches through genetic counseling in affected consanguineous families. All together, these findings highlight the specific nature of highly consanguineous populations and confirm the importance of unraveling the molecular basis of genetic diseases in this context. Besides providing a better fundamental knowledge of novel pathways, their study is improving diagnosis strategies and appropriate care.
    Sprache Englisch
    Erscheinungsdatum 2017
    Erscheinungsland Switzerland
    Dokumenttyp Journal Article ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2017.00737
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  6. Artikel ; Online: Diagnostic challenge in a series of eleven patients with hyper IgE syndromes.

    Yaakoubi, Roukaya / Mekki, Najla / Ben-Mustapha, Imen / Ben-Khemis, Leila / Bouaziz, Asma / Ben Fraj, Ilhem / Ammar, Jamel / Hamzaoui, Agnès / Turki, Hamida / Boussofara, Lobna / Denguezli, Mohamed / Haddad, Samir / Ouederni, Monia / Bejaoui, Mohamed / Chan, Koon Wing / Lau, Yu Lung / Mellouli, Fethi / Barbouche, Mohamed-Ridha / Ben-Ali, Meriem

    Frontiers in immunology

    2023  Band 13, Seite(n) 1057679

    Abstract: Hyper IgE syndromes (HIES) is a heterogeneous group of Inborn Errors of Immunity characterized by eczema, recurrent skin and lung infections associated with eosinophilia and elevated IgE levels. Autosomal dominant HIES caused by loss of function ... ...

    Abstract Hyper IgE syndromes (HIES) is a heterogeneous group of Inborn Errors of Immunity characterized by eczema, recurrent skin and lung infections associated with eosinophilia and elevated IgE levels. Autosomal dominant HIES caused by loss of function mutations in Signal transducer and activator of transcription 3 (
    Mesh-Begriff(e) Humans ; Job Syndrome/diagnosis ; Job Syndrome/genetics ; Guanine Nucleotide Exchange Factors ; Skin ; Phenotype ; Eosinophilia/complications
    Chemische Substanzen Guanine Nucleotide Exchange Factors ; DOCK8 protein, human
    Sprache Englisch
    Erscheinungsdatum 2023-01-10
    Erscheinungsland Switzerland
    Dokumenttyp Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2022.1057679
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  7. Artikel ; Online: Regulatory T-cell dysfunction and cutaneous exposure to Staphylococcus aureus underlie eczema in DOCK8 deficiency.

    Wilkie, Hazel / Das, Mrinmoy / Pelovitz, Tyler / Bainter, Wayne / Woods, Brian / Alasharee, Mohammed / Sobh, Ali / Baris, Safa / Eltan, Sevgi Bilgic / Al-Herz, Waleed / Barbouche, Mohamed-Ridha / Ben-Mustapha, Imen / Ben-Ali, Meriem / Sallam, Mohamed T H / Awad, Amany / Lotfy, Sohilla / El Marsafy, Aisha / Ezzelarab, Moushira / Farrar, Michael /
    Schmidt, Brigitta A R / NandyMazumdar, Monali / Guttman-Yassky, Emma / Sheets, Anthony / Vidic, Katie Maria / Murphy, George / Schlievert, Patrick M / Chou, Janet / Leyva-Castillo, Juan Manuel / Janssen, Erin / Timilshina, Maheshwor / Geha, Raif S

    The Journal of allergy and clinical immunology

    2024  

    Abstract: Background: Dedicator of cytokinesis 8 (DOCK8)-deficient patients have severe eczema, elevated IgE, and eosinophilia, features of atopic dermatitis (AD).: Objective: We sought to understand the mechanisms of eczema in DOCK8 deficiency.: Methods: ... ...

    Abstract Background: Dedicator of cytokinesis 8 (DOCK8)-deficient patients have severe eczema, elevated IgE, and eosinophilia, features of atopic dermatitis (AD).
    Objective: We sought to understand the mechanisms of eczema in DOCK8 deficiency.
    Methods: Skin biopsy samples were characterized by histology, immunofluorescence microscopy, and gene expression. Skin barrier function was measured by transepidermal water loss. Allergic skin inflammation was elicited in mice by epicutaneous sensitization with ovalbumin (OVA) or cutaneous application of Staphylococcus aureus.
    Results: Skin lesions of DOCK8-deficient patients exhibited type 2 inflammation, and the patients' skin was colonized by Saureus, as in AD. Unlike in AD, DOCK8-deficient patients had a reduced FOXP3:CD4 ratio in their skin lesions, and their skin barrier function was intrinsically intact. Dock8
    Conclusion: Treg cell dysfunction increases susceptibility to allergic skin inflammation in DOCK8 deficiency and synergizes with cutaneous exposure to Saureus to drive eczema in DOCK8 deficiency.
    Sprache Englisch
    Erscheinungsdatum 2024-01-05
    Erscheinungsland United States
    Dokumenttyp Journal Article
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2023.12.020
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  8. Artikel ; Online: Defective glycosylation leads to defective gp130-dependent STAT3 signaling in PGM3-deficient patients.

    Ben-Ali, Meriem / Ben-Khemis, Leila / Mekki, Najla / Yaakoubi, Roukaya / Ouni, Rym / Benabdessalem, Chaouki / Ben-Mustapha, Imen / Barbouche, Mohamed-Ridha

    The Journal of allergy and clinical immunology

    2018  Band 143, Heft 4, Seite(n) 1638–1640.e2

    Mesh-Begriff(e) Cytokine Receptor gp130/metabolism ; Glycosylation ; Humans ; Phosphoglucomutase/deficiency ; Phosphoglucomutase/genetics ; Primary Immunodeficiency Diseases/genetics ; Primary Immunodeficiency Diseases/immunology ; Primary Immunodeficiency Diseases/metabolism ; STAT3 Transcription Factor/metabolism ; Signal Transduction/immunology
    Chemische Substanzen STAT3 Transcription Factor ; STAT3 protein, human ; Cytokine Receptor gp130 (133483-10-0) ; PGM3 protein, human (EC 5.4.2.2) ; Phosphoglucomutase (EC 5.4.2.2)
    Sprache Englisch
    Erscheinungsdatum 2018-12-19
    Erscheinungsland United States
    Dokumenttyp Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2018.12.987
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  9. Artikel ; Online: Adénite granulomateuse révélant un déficit en récepteur de l’IL12.

    Kamoun, Fatma / Sfaihi, Lamia / Ben Mustapha, Imen / Ben Ameur, Salma / Barbouche, Mohamed Ridha / Hachicha, Mongia

    Presse medicale (Paris, France : 1983)

    2017  Band 46, Heft 3, Seite(n) 346–348

    Titelübersetzung Granulomatous lymphadenitis revealing a deficiency in receptor IL12.
    Sprache Französisch
    Erscheinungsdatum 2017-03
    Erscheinungsland France
    Dokumenttyp Letter
    ZDB-ID 120943-7
    ISSN 2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
    ISSN (online) 2213-0276
    ISSN 0032-7867 ; 0755-4982 ; 0301-1518
    DOI 10.1016/j.lpm.2016.11.017
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  10. Artikel: Cutaneous Manifestations of Primary Immunodeficiency Diseases in Tunisian Children.

    Dhouib, Naouel Guirat / Ben Khaled, Monia / Ouederni, Monia / Ben-Mustapha, Imen / Kouki, Ridha / Besbes, Habib / Barbouche, Mohamed Ridha / Mellouli, Fethi / Bejaoui, Mohamed

    Mediterranean journal of hematology and infectious diseases

    2018  Band 10, Heft 1, Seite(n) e2018065

    Abstract: Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic ... ...

    Abstract Skin manifestations are frequent among patients with primary immunodeficiency diseases (PIDs). Their prevalence varies according to the type of immunodeficiency. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of PIDs among Tunisian children. We conducted a prospective study on two hundred and ninety children with immune deficiency. Demographic details (including age, sex, and consanguinity) with personal and family history were recorded. Special attention was paid to cutaneous manifestations. Dermatological involvements were grouped according to the etiology of their most prominent sign. Cutaneous manifestations were found in 164 patients (56.5%). They revealed the diagnosis of PIDs in 71 patients (24.5 %). The mean age at presentation was 21 months. Overall the most prominent cutaneous alterations were infectious. They accounted for 106 cases (36.55%). The most prevalent causes of cutaneous infections were bacterial: 93 cases (32.06%). Immuno-allergic skin diseases were among the common findings in our study. These include eczematous dermatitis found in 62 cases (21.38%). Malignancy related PIDs was seen in a boy with Wiskott Aldrich syndrome. He developed Kaposi's sarcoma at the age of 14 months. Cutaneous changes are common among children with PIDs. In pediatric patients with failure to thrive, chronic refractory systemic manifestations often present in other family members, recurrent cutaneous infections unresponsive to adequate therapy, atypical forms of eczematous dermatitis or unusual features should arouse the suspicion of PIDs and prompt specialized immunologic consultation should be made.
    Sprache Englisch
    Erscheinungsdatum 2018-11-01
    Erscheinungsland Italy
    Dokumenttyp Journal Article
    ZDB-ID 2674750-9
    ISSN 2035-3006
    ISSN 2035-3006
    DOI 10.4084/MJHID.2018.065
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