The wiskott-aldrich syndrome

HD Ochs, AJ Thrasher - Journal of Allergy and Clinical Immunology, 2006 - Elsevier
The Wiskott-Aldrich syndrome (WAS) is a rare X-linked disorder with variable clinical
phenotypes that correlate with the type of mutations in the WAS protein (WASP) gene …

β2 Integrins—multi-functional leukocyte receptors in health and disease

M Bednarczyk, H Stege, S Grabbe, M Bros - International journal of …, 2020 - mdpi.com
β2 integrins are heterodimeric surface receptors composed of a variable α (CD11a-CD11d)
and a constant β (CD18) subunit and are specifically expressed by leukocytes. The α …

Loss of the Arp2/3 complex component ARPC1B causes platelet abnormalities and predisposes to inflammatory disease

WHA Kahr, FG Pluthero, A Elkadri, N Warner… - Nature …, 2017 - nature.com
Abstract Human actin-related protein 2/3 complex (Arp2/3), required for actin filament
branching, has two ARPC1 component isoforms, with ARPC1B prominently expressed in …

Cytoskeletal actin dynamics shape a ramifying actin network underpinning immunological synapse formation

M Fritzsche, RA Fernandes, VT Chang… - Science …, 2017 - science.org
T cell activation and especially trafficking of T cell receptor microclusters during
immunological synapse formation are widely thought to rely on cytoskeletal remodeling …

Interfacial actin protrusions mechanically enhance killing by cytotoxic T cells

F Tamzalit, MS Wang, W Jin, M Tello-Lafoz… - Science …, 2019 - science.org
Cytotoxic T lymphocytes (CTLs) kill by forming immunological synapses with target cells and
secreting toxic proteases and the pore-forming protein perforin into the intercellular space …

Autoinflammatory periodic fever, immunodeficiency, and thrombocytopenia (PFIT) caused by mutation in actin-regulatory gene WDR1

ASI Standing, D Malinova, Y Hong, J Record… - Journal of Experimental …, 2017 - rupress.org
The importance of actin dynamics in the activation of the inflammasome is becoming
increasingly apparent. IL-1β, which is activated by the inflammasome, is known to be central …

Large‐sized graphene oxide nanosheets increase DC–T‐cell synaptic contact and the efficacy of DC vaccines against SARS‐CoV‐2

Q Zhou, H Gu, S Sun, Y Zhang, Y Hou, C Li… - Advanced …, 2021 - Wiley Online Library
Dendritic cell (DC) vaccines are used for cancer and infectious diseases, albeit with limited
efficacy. Modulating the formation of DC–T‐cell synapses may greatly increase their efficacy …

Self-organizing actin patterns shape membrane architecture but not cell mechanics

M Fritzsche, D Li, H Colin-York, VT Chang… - Nature …, 2017 - nature.com
Cell-free studies have demonstrated how collective action of actin-associated proteins can
organize actin filaments into dynamic patterns, such as vortices, asters and stars. Using …

Multiple actin networks coordinate mechanotransduction at the immunological synapse

D Blumenthal, JK Burkhardt - Journal of Cell Biology, 2020 - rupress.org
Activation of naive T cells by antigen-presenting cells (APCs) is an essential step in
mounting an adaptive immune response. It is known that antigen recognition and T cell …

Understanding immunoactinopathies: A decade of research on WAS gene defects

RC Vieira, LG Pinho… - Pediatric Allergy and …, 2023 - Wiley Online Library
Immunoactinopathies caused by mutations in actin‐related proteins are a growing group of
inborn errors of immunity (IEI). Immunoactinopathies are caused by a dysregulated actin …