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〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥

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1. BおよびTリンパ球の正常な発生 normal b and t lymphocyte development
2. リードスタンバーグ細胞およびホジキンリンパ腫の病因 the reed sternberg cell and the pathogenesis of hodgkin lymphoma

English Journal

IRF4 deficiency leads to altered BCR signaling revealed by enhanced PI3K pathway, decreased SHIP expression and defected cytoskeletal responses.

Budzyńska PM1,2, Niemelä M1, Sarapulov AV3, Kyläniemi MK1, Nera KP1, Junttila S4, Laiho A4, Mattila PK3, Alinikula J1, Lassila O1.
Scandinavian journal of immunology.Scand J Immunol.2015 Jul 14. doi: 10.1111/sji.12343. [Epub ahead of print]
The graded expression of transcription factor Interferon Regulatory Factor 4 (IRF4) regulates B cell development and is critical for plasma cell differentiation. However, the mechanisms, by which IRF4 elicits its crucial tasks, are largely unknown. To characterize the molecular targets of IRF4 in B
PMID 26173778

Enteroviral Infection in a Patient with BLNK Adaptor Protein Deficiency.

NaserEddin A1, Shamriz O, Keller B, Alzyoud RM, Unger S, Fisch P, Prus E, Berkun Y, Averbuch D, Shaag A, Wahadneh AM, Conley ME, Warnatz K, Elpeleg O, Stepensky P.
Journal of clinical immunology.J Clin Immunol.2015 May;35(4):356-60. doi: 10.1007/s10875-015-0164-2. Epub 2015 Apr 17.
B-cell linker (BLNK) protein is a non-redundant adaptor molecule in the signaling pathway activated by (pre) B-cell antigen receptor signals. We present two siblings with a homozygous deleterious frameshift mutation in BLNK, resulting in a block of B cell development in the bone marrow at the preB1
PMID 25893637

The autoinhibitory C-terminal SH2 domain of phospholipase C-γ2 stabilizes B cell receptor signalosome assembly.

Wang J1, Sohn H1, Sun G2, Milner JD2, Pierce SK3.
Science signaling.Sci Signal.2014 Sep 16;7(343):ra89. doi: 10.1126/scisignal.2005392.
The binding of antigen to the B cell receptor (BCR) stimulates the assembly of a signaling complex (signalosome) composed initially of the kinases Lyn, spleen tyrosine kinase (Syk), and Bruton's tyrosine kinase (Btk), as well as the adaptor protein B cell linker (BLNK). Together, these proteins recr
PMID 25227611

Related Links

BLNK Deficiency | SpringerLink

B-cell linker protein (BLNK) deficiency (OMIM #613502) is a rare primary immunodeficiency characterized by reduced serum levels of all immunoglobulin classes in the absence of peripheral B cells (peripheral B cells <2%). To date, a ...

Deficiency of BLNK hampers PLC-γ2 phosphorylation and ...

As stated above, BLNK-deficient mice have a block in B-cell development at the pre-B-cell stage, but the block is incomplete, and a small number of mature B cells are still present in the periphery. 23 – 26 This suggests that other

BLNK B cell linker - Gene - GTR - NCBI

Deficiency in this protein has also been shown in some cases of pre-B acute lymphoblastic leukemia. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, May 2012]