The NF-kappa B/Rel and I kappa B proteins are important regulators of
lymphocyte activation and gene expression. We have identified a rearra
ngement of the NFKB-2 gene in the HUT 78 human cutaneous T-cell leukem
ia (CTCL) line. cDNA and genomic DNA sequence predicted the presence o
f a truncated 80 kD NFKB-2 precursor protein (p80HT), instead of the n
ormal p100 protein. No wild-type allele was identified. Elevated level
s of two aberrantly sized RNAs were detected, and high levels of p80HT
and processed p52 protein were present in HUT 78 cell nuclei. The p52
protein bound to a palindromic KB DNA motif, however p80HT did not. R
earrangement of the NFKB-2 gene was also detected in DNA from two pati
ents with CTCL. Rearrangement and overexpression of the NFKB-2 gene ma
y contribute to the genesis of a subset of T-cell malignancies.