A Novel Imprinted Gene, KCNQ1DN, within the WT2 Critical Region of Human Chromosome 11p15.5 and Its Reduced Expression in Wilms' Tumors

WT2 is defined by a maternal-specific loss of heterozygosity on human chromosome 11p15.5 in Wilms' and other embryonal tumors. Therefore, the imprinted genes in this region are candidates for involvement in Wilms' tumorigenesis. We now report a novel imprinted gene, KCNQ1DN (KCNQ1 downstre...

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Veröffentlicht in:Journal of biochemistry (Tokyo) 2000-11, Vol.128 (5), p.847-853
Hauptverfasser: Xin, Zhenghan, Soejima, Hidenobu, Higashimoto, Ken, Yatsuki, Hitomi, Zhu, Xike, Satoh, Yuji, Masaki, Zenjiro, Kaneko, Yasuhiko, Jinno, Yoshihiro, Fukuzawa, Ryuji, Hata, Jun-ichi, Mukai, Tsunehiro
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container_issue 5
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container_title Journal of biochemistry (Tokyo)
container_volume 128
creator Xin, Zhenghan
Soejima, Hidenobu
Higashimoto, Ken
Yatsuki, Hitomi
Zhu, Xike
Satoh, Yuji
Masaki, Zenjiro
Kaneko, Yasuhiko
Jinno, Yoshihiro
Fukuzawa, Ryuji
Hata, Jun-ichi
Mukai, Tsunehiro
description WT2 is defined by a maternal-specific loss of heterozygosity on human chromosome 11p15.5 in Wilms' and other embryonal tumors. Therefore, the imprinted genes in this region are candidates for involvement in Wilms' tumorigenesis. We now report a novel imprinted gene, KCNQ1DN (KCNQ1 downstream neighbor). This gene is located between p57KIP2 and KuLQT1 (KCNQ1) of 11p15.5 within the WT2 critical region. KCNQ1DN is imprinted and expressed from the maternal allele. We examined the expression of KCNQ1DN in Wilms' tumors. Seven of eighteen (39%) samples showed no expression. In contrast, other maternal imprinted genes in this region, including p57KIP2, IMPT1, and IPL exhibited almost normal expression in these samples, although some samples expressed IGF2 biallelically. These results suggest that KCNQ1DN existing far from the H19øIGF2 region may play some role in Wilms' tumorigenesis along with IGF2.
doi_str_mv 10.1093/oxfordjournals.jbchem.a022823
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Therefore, the imprinted genes in this region are candidates for involvement in Wilms' tumorigenesis. We now report a novel imprinted gene, KCNQ1DN (KCNQ1 downstream neighbor). This gene is located between p57KIP2 and KuLQT1 (KCNQ1) of 11p15.5 within the WT2 critical region. KCNQ1DN is imprinted and expressed from the maternal allele. We examined the expression of KCNQ1DN in Wilms' tumors. Seven of eighteen (39%) samples showed no expression. In contrast, other maternal imprinted genes in this region, including p57KIP2, IMPT1, and IPL exhibited almost normal expression in these samples, although some samples expressed IGF2 biallelically. 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subjects Chromosome Mapping
Chromosomes, Human, Pair 11
CpG island
Female
Gene Expression Regulation, Neoplastic
Gene Library
Genomic Imprinting
Humans
imprinted genes
KCNQ Potassium Channels
KCNQ1 Potassium Channel
KCNQ1DN
Loss of Heterozygosity
Male
Molecular Sequence Data
Potassium Channels - genetics
Potassium Channels, Voltage-Gated
Testis - chemistry
Wilms Tumor - genetics
Wilms' tumors
title A Novel Imprinted Gene, KCNQ1DN, within the WT2 Critical Region of Human Chromosome 11p15.5 and Its Reduced Expression in Wilms' Tumors
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