Perforin and granzymes: function, dysfunction and human pathology

I Voskoboinik, JC Whisstock, JA Trapani - Nature Reviews Immunology, 2015 - nature.com
A defining property of cytotoxic lymphocytes is their expression and regulated secretion of
potent toxins, including the pore-forming protein perforin and serine protease granzymes …

The genetics of macrophage activation syndrome

GS Schulert, RQ Cron - Genes & Immunity, 2020 - nature.com
Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphohistiocytosis
(HLH), is a cytokine storm syndrome associated with multi-organ system dysfunction and …

Tumor immune evasion arises through loss of TNF sensitivity

CJ Kearney, SJ Vervoort, SJ Hogg… - Science …, 2018 - science.org
Immunotherapy has revolutionized outcomes for cancer patients, but the mechanisms of
resistance remain poorly defined. We used a series of whole-genome clustered regularly …

Whole-exome sequencing reveals mutations in genes linked to hemophagocytic lymphohistiocytosis and macrophage activation syndrome in fatal cases of H1N1 …

GS Schulert, M Zhang, N Fall, A Husami… - The Journal of …, 2016 - academic.oup.com
Abstract Background. Severe H1N1 influenza can be lethal in otherwise healthy individuals
and can have features of reactive hemophagocytic lymphohistiocytosis (HLH). HLH is …

Low-frequency and rare-coding variation contributes to multiple sclerosis risk

M Mitrovič, NA Patsopoulos, AH Beecham… - Cell, 2018 - cell.com
Multiple sclerosis is a complex neurological disease, with∼ 20% of risk heritability
attributable to common genetic variants, including> 230 identified by genome-wide …

MAIT cells regulate NK cell-mediated tumor immunity

EV Petley, HF Koay, MA Henderson, K Sek… - Nature …, 2021 - nature.com
The function of MR1-restricted mucosal-associated invariant T (MAIT) cells in tumor
immunity is unclear. Here we show that MAIT cell-deficient mice have enhanced NK cell …

Highways to hell: Mechanism-based management of cytokine storm syndromes

SW Canna, RQ Cron - Journal of Allergy and Clinical Immunology, 2020 - Elsevier
Since the first textbook devoted to cytokine storm syndromes (CSSs) was published in 2019,
the world has changed dramatically and the term's visibility has broadened. Herein, we …

Beyond target cell death–granzyme serine proteases in health and disease

S Nüssing, VR Sutton, JA Trapani, IA Parish - Molecular Aspects of …, 2022 - Elsevier
Granzymes are a family of small (∼ 32 kDa) serine proteases with a range of substrate
specificities that are stored in, and released from, the cytoplasmic secretory vesicles …

Antagonism of IAPs enhances CAR T-cell efficacy

J Michie, PA Beavis, AJ Freeman, SJ Vervoort… - Cancer immunology …, 2019 - AACR
Chimeric antigen receptor (CAR) T-cell therapy has proven successful in the treatment of
hematological malignancies, notably acute lymphoblastic leukemia and B-cell lymphoma …

The multifaceted immunology of cytokine storm syndrome

PY Lee, RQ Cron - The Journal of Immunology, 2023 - journals.aai.org
Cytokine storm syndromes (CSSs) are potentially fatal hyperinflammatory states that share
the underpinnings of persistent immune cell activation and uninhibited cytokine production …