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Nigel Sharfe

Explore the profile of Nigel Sharfe including associated specialties, affiliations and a list of published articles. Areas
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Articles 22
Citations 395
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Recent Articles
1.
Merico D, Sharfe N, Dadi H, Thiruvahindrapuram B, de Rijke J, Dahi Z, et al.
NPJ Genom Med . 2025 Jan; 10(1):2. PMID: 39805825
Maturation of αβ lineage T cells in the thymus relies on the formation and cell surface expression of a pre-T cell receptor (TCR) complex, composed of TCRβ chain and pre-TCRα...
2.
Shiraishi A, Uygun V, Sharfe N, Beldar S, Sun M, Dadi H, et al.
Blood . 2023 Feb; 141(26):3226-3230. PMID: 36745868
No abstract available.
3.
Sharfe N, Dalal I, Naghdi Z, Lefaudeux D, Vong L, Dadi H, et al.
J Autoimmun . 2022 Nov; 137:102946. PMID: 36402602
Background: Genetic aberrations in the NFκB pathway lead to primary immunodeficiencies with various degrees of severity. We previously demonstrated that complete ablation of the RelB transcription factor, a key component...
4.
Scott O, Sharfe N, Dadi H, Vong L, Garkaby J, Abrego Fuentes L, et al.
Front Immunol . 2022 Feb; 13:801832. PMID: 35126392
Background: STAT1 gain-of-function (GOF) is a primary immune dysregulatory disorder marked by wide infectious predisposition (most notably chronic mucocutaneous Candidiasis), autoimmunity, vascular disease and malignant predisposition. While atopic features have...
5.
Mandola A, Sharfe N, Nagdi Z, Dadi H, Vong L, Merico D, et al.
J Allergy Clin Immunol . 2020 Sep; 147(2):727-733.e2. PMID: 32980423
Background: Genetic faults in several components of the nuclear factor-κB pathway cause immunodeficiency. Most defects lead to combined immunodeficiency with a range of severity. Heterozygous mutations in NFKB1 were associated...
6.
Nahum A, Sharfe N, Broides A, Dadi H, Naghdi Z, Mandola A, et al.
J Allergy Clin Immunol . 2019 Nov; 145(3):1011-1015.e6. PMID: 31778705
No abstract available.
7.
Ovadia A, Sharfe N, Hawkins C, Laughlin S, Roifman C
NPJ Genom Med . 2018 Aug; 3:23. PMID: 30131873
Signal transducer and activator of transcription 1 (STAT1) regulates multiple biological processes downstream of a variety of cytokine receptors in many cell types. Heterozygous gain-of-function (GOF) mutations in STAT1 have...
8.
Sharfe N, Karanxha A, Dadi H, Merico D, Chitayat D, Herbrick J, et al.
J Allergy Clin Immunol . 2017 Nov; 142(2):618-629. PMID: 29180244
Background: We previously reported a novel syndrome characterized by combined immunodeficiency associated with severe developmental defects-subsequently known as Roifman-Chitayat syndrome (RCS; OMIM 613328). Linkage analysis identified 2 disease-associated loci. Objectives:...
9.
Dadi H, Jones T, Merico D, Sharfe N, Ovadia A, Schejter Y, et al.
J Allergy Clin Immunol . 2017 Aug; 141(5):1818-1830.e2. PMID: 28826773
Background: Combined immunodeficiency (CID) is a T-cell defect frequently presenting with recurrent infections, as well as associated immune dysregulation manifesting as autoimmunity or allergic inflammation. Objective: We sought to identify...
10.
Sharfe N, Merico D, Karanxha A, Macdonald C, Dadi H, Ngan B, et al.
J Autoimmun . 2015 Sep; 65:90-100. PMID: 26385063
Multiple receptors that control cell growth and inflammation activate the NFκB pathway that comprises of two pathways. Dysfunction of the classical pathway leads to impaired adaptive and innate immunity in...