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BACKGROUND
Non-T cell activation linker (NTAL), a transmembrane adaptor protein, is
also designated membrane-associated adapter molecule, Williams-Beuren
syndrome chromosome region 15 protein or LAB (linker of activated B cells).
NTAL is present in membrane microdomains (rafts) of B cells, NK cells and
myeloid cells, and in monocytes and mast cells, but not in resting T lymphocytes.
NTAL becomes rapidly tyrosine-phosphorylated upon cross-linking of
the B cell receptor (BCR) or of high-affinity Fc γ and Fc ε receptors of myeloid
cells and then associates with the cytoplasmic signaling molecules. NTAL is
highly expressed in spleen, lymph node germinal centers and peripheral blood
lymphocytes. Defects in the gene encoding for NTAL may cause the musculoskeletal
and cardiovascular abnormalities that characterize the rare developmental
disorder Williams-Beuren syndrome (WBS).

 

REFERENCES
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V. and Koop, B.F. 2000. Comparative genomic sequence analysis of the
Williams syndrome region (LIMK1-RFC2) of human chromosome 7q11.23.
Mamm. Genome 11: 890-898.
2. Janssen, E., Zhu, M., Zhang, W., Koonpaew, S. and Zhang, W. 2003. LAB:
a new membrane-associated adaptor molecule in B cell activation. Nat.
Immunol. 4: 117-123.
3. Tkaczyk, C., Horejsi, V., Iwaki, S., Draber, P., Samelson, L.E., Satterthwaite,
A.B., Nahm, D.H., Metcalfe, D.D. and Gilfillan, A.M. 2004. NTAL phosphorylation
is a pivotal link between the signaling cascades leading to human
mast cell degranulation following Kit activation and Fc ε RI aggregation.
Blood 104: 207-214.
4. Koonpaew, S., Janssen, E., Zhu, M. and Zhang, W. 2004. The importance
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markers. Blood 107: 213-221.