THE X-CHROMOSOMAL HUMAN BIGLYCAN GENE BGN IS SUBJECT TO X-INACTIVATION BUT IS TRANSCRIBED LIKE AN X-Y HOMOLOGOUS GENE

被引：46

作者：

GEERKENS, C ^{[1
]}

VETTER, U ^{[1
]}

JUST, W ^{[1
]}

FEDARKO, NS ^{[1
]}

FISHER, LW ^{[1
]}

YOUNG, MF ^{[1
]}

TERMINE, JD ^{[1
]}

ROBEY, PG ^{[1
]}

WOHRLE, D ^{[1
]}

VOGEL, W ^{[1
]}

机构：

[1] NIDR,BONE RES BRANCH,BETHESDA,MD 20892

来源：

HUMAN GENETICS | 1995年 / 96卷 / 01期

关键词：

D O I：

10.1007/BF00214185

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

We report the mRNA and protein expression levels of human biglycan (BGN) in patients with different numbers of sex chromosomes. BGN maps to the distal long arm of the X chromosome, band Xq28, near the second pseudoautosomal region. BGN expression levels are reduced in 45,X Turner patients and increased in patients with additional sex chromosomes. This is suggestive of a pseudoautosomal gene or a gene that escapes X inactivation and that has an active Y chromosomal copy. However, we also provide evidence from hybrid cell lines that BGN is subject to X inactivation and that there is no homolog on the Y chromosome. This evidence excludes an escape from X inactivation. Moreover, additional Y chromosomes increase BGN expression levels, despite the absence of a Y chromosomal BGN gene. Therefore, another explanation has to be invoked. The ''pseudoautosomal expression'' of BGN may be attributed to a gene or genes that escape X inactivation and that regulate the transcriptional activity of BGN. This is the first report concerning an X chromosomal gene that does not show the conventional correlation between gene dosage and expression rate known from other X chromosomal genes.

引用

页码：44 / 52

页数：9

共 40 条

[1] A NOVEL X-GENE WITH A WIDELY TRANSCRIBED Y-LINKED HOMOLOG ESCAPES X-INACTIVATION IN MOUSE AND HUMAN [J].