Details

Autor(en) / Beteiligte

• Moran, Imogen
• Avery, Danielle T.
• Payne, Kathryn
• Lenthall, Helen
• Davies, E. Graham
• Burns, Siobhan
• Ip, Winnie
• Oleastro, Matÿfffedas M.
• Reisli, Ismail
• Guner, Sukru
• Keles, Sevgi
• Notarangelo, Luigi
• Deenick, Elissa K.
• Goodnow, Christopher C.
• Zahra, David
• Brink, Robert
• Wong, Melanie
• Tangye, Stuart G.
• Ma, Cindy S.
• Phan, Tri Giang

Titel

B cell–intrinsic requirement for STK4 in humoral immunity in mice and human subjects

Ist Teil von

• Journal of allergy and clinical immunology, 2019-06, Vol.143 (6), p.2302-2305

Ort / Verlag

St. Louis: Elsevier Inc

Erscheinungsjahr

2019

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• To the Editor: Biallelic loss-of-function mutations in serine-threonine kinase 4 (STK4), also known as mammalian sterile 20-like 1 (MST1), are associated with a combined immunodeficiency characterized by recurrent bacterial, fungal, and viral infections.1-6 Most patients have intermittent neutropenia, T and B lymphopenia, and, paradoxically, specific antibody defects despite the reported presence of increased levels of serum IgG, IgA, and IgE antibodies and antibody-mediated autoimmune cytopenias.1-6 Although there have been numerous studies of neutrophil, macrophage, dendritic cell, and T-cell function in Stk4 knockout mice,7-9,E1,E2 the nature and basis of the underlying B-cell dysregulation in STK4-deficient patients remains poorly characterized. [...]there were no differences in B-cell receptor–mediated upregulation of CD69 and CD86 after in vitro stimulation with cognate antigen of STK4-deficient mouse B cells (Fig 2, D). Interestingly, despite the impaired specific antibody secretion in mice with Stk4Y88del/Y88del B cells, STK4 deficiency did not quantitatively affect the ability of these SWHEL B cells to generate plasma cells in vivo (Fig 2, I-K). [...]similar to B cells from STK4-deficient patients, Stk4Y88del/Y88del B cells are able to differentiate into plasma cells, but these plasma cells do not secrete adequate amounts of specific antibody.