Molecules responsible for embryogenesis are often involved in
tumorigenesis. Recent exhaustive
cDNA microarray analyses in human
neoplasms expanded knowledge of such molecules.
Hepatocyte nuclear factor-1beta (HNF-1beta) is a homeobox
transcription factor that functions as a homodimer or heterodimer with
HNF-1alpha. In contrast to
HNF-1alpha,
HNF-1beta is very weakly expressed in the liver and is commonly expressed in the kidneys. During human embryonic stage,
HNF-1beta plays an important role in organogenesis, especially of the urogenital system. In the human fetus,
HNF-1beta expression is common in mesonephric duct derivatives and metanephros (permanent kidneys).
HNF-1beta germline mutations cause malformations of these structures. Recent microarray analyses have disclosed that
HNF-1beta is aberrantly up-regulated in clear cell
carcinoma of the ovary, which is a
carcinoma of müllerian nature, but which was initially misnamed "
mesonephroma".
HNF-1beta is also expressed in ovarian
endometriosis, which is a probable origin of clear cell
carcinoma. On the other hand,
HNF-1beta is down-regulated in
renal neoplasms, such as chromophobe cell
carcinoma. In this review, we first summarize
HNF-1beta expression in the developing urogenital system of the human embryo. Then, we describe the
HNF-1beta status in human
urogenital neoplasms and discuss its role in
tumorigenesis.