28 research outputs found
Mutations affecting the actin regulator WD repeat–containing protein 1 lead to aberrant lymphoid immunity
Background: The actin-interacting protein WD repeat–containing protein 1 (WDR1) promotes cofilin-dependent actin filament turnover. Biallelic WDR1 mutations have been identified recently in an immunodeficiency/autoinflammatory syndrome with aberrant morphology and function of myeloid cells. Objective: Given the pleiotropic expression of WDR1, here we investigated to what extent it might control the lymphoid arm of the immune system in human subjects. Methods: Histologic and detailed immunologic analyses were performed to elucidate the role of WDR1 in the development and function of B and T lymphocytes. Results: Here we identified novel homozygous and compound heterozygous WDR1 missense mutations in 6 patients belonging to 3 kindreds who presented with respiratory tract infections, skin ulceration, and stomatitis. In addition to defective adhesion and motility of neutrophils and monocytes, WDR1 deficiency was associated with aberrant T-cell activation and B-cell development. T lymphocytes appeared to develop normally in the patients, except for the follicular helper T-cell subset. However, peripheral T cells from the patients accumulated atypical actin structures at the immunologic synapse and displayed reduced calcium flux and mildly impaired proliferation on T-cell receptor stimulation. WDR1 deficiency was associated with even more severe abnormalities of the B-cell compartment, including peripheral B-cell lymphopenia, paucity of B-cell progenitors in the bone marrow, lack of switched memory B cells, reduced clonal diversity, abnormal B-cell spreading, and increased apoptosis on B-cell receptor/Toll-like receptor stimulation. Conclusion: Our study identifies a novel role for WDR1 in adaptive immunity, highlighting WDR1 as a central regulator of actin turnover during formation of the B-cell and T-cell immunologic synapses
hs578T
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Each file is a Multi-plane tiff, containing three fluorescence channels:
Channel 1 = DAPI (Sigma)
Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
File names refer to [row][column]-[field
ZR75.1
Files are 16-bit tiffs (which means they will appear black if opened in Preview, Photoshop, or similar program). Each file is a Multi-plane tiff, containing three fluorescence channels:
Channel 1 = DAPI (Sigma)
Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
File names refer to [row][column]-[field]
See ReadMe file for culture and staining information
HCC1954
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Each file is a Multi-plane tiff, containing three fluorescence channels:
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Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
File names refer to [row][column]-[field
SKBR3
Files are 16-bit tiffs (which means they will appear black if opened in Preview, Photoshop, or similar program). Each file is a Multi-plane tiff, containing three fluorescence channels:
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Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
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See ReadMe file for culture and staining information
AU565
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Each file is a Multi-plane tiff, containing three fluorescence channels:
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Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
File names refer to [row][column]-[field
BT474
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Each file is a Multi-plane tiff, containing three fluorescence channels:
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Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
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HeLa
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Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
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MCF12A-no EGF
Files are 16-bit tiffs (which means they will appear black if opened in Preview, Photoshop, or similar program).
Each file is a Multi-plane tiff, containing three fluorescence channels:
Channel 1 = DAPI (Sigma)
Channel 2 = NF-kappaB (anti-p65; Abcam ab16502 / Alexa-488 anti-rabbit; Invitrogen)
Channel 3 = DHE (dihydroethidium, hydroethidine; Sigma)
File names refer to [row][column]-[field