ZAP-70 in B cell malignancies.

Abstract:

:ZAP-70 has emerged as a protein of potential prognostic importance in chronic lymphocytic leukemia (CLL) following gene expression profiling which compared the 2 well established prognostic sub-sets, those with unmutated and mutated IgVH genes. This protein tyrosine kinase (PTK), known to be of importance in T and NK cell signaling but absent in normal peripheral B cells, is expressed in the majority of the poorer prognosis unmutated CLL and absent in most cases with mutated IgVH genes. ZAP-70 has been shown to be functionally important in the CLL cases in which it is expressed; it is also important in B cell development in mice and there is preliminary evidence for its expression in human B cell progenitors and activated B cells. Whether its expression in a sub-set of CLL cases is a result of a more activated cell type or a reflection of the stage of maturation of the transforming event(s) in CLL is open to debate. ZAP-70 is expressed in a minority of other B cell tumors but correlation with IgVH gene mutational status is lacking. The problems with ZAP-70 measurement, which has yet to be standardized, are reviewed together with its current status as a prognostic marker in CLL.

journal_name

Leuk Lymphoma

journal_title

Leukemia & lymphoma

authors

Orchard J,Ibbotson R,Best G,Parker A,Oscier D

doi

10.1080/09638280500260079

keywords:

subject

Has Abstract

pub_date

2005-12-01 00:00:00

pages

1689-98

issue

12

eissn

1042-8194

issn

1029-2403

pii

Q61UW752554K001T

journal_volume

46

pub_type

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