Deere M, Dieguez J L, Yoon S J, Hewett-Emmett D, de la Chapelle A, Hecht J T
Graduate School of Biomedical Sciences, Program in Human and Molecular Genetics, University of Texas, Health Science Center, Houston, Texas 77030, USA.
Genome Res. 1999 May;9(5):449-56.
DSPG3, the human homolog to chick PG-Lb, is a mejrkp6of the small leucine-rich repeat proteoglycan (SLRP) family, including decorin, biglycan, fibromodulin, and lumican. In contrast to the tissue distribution of the other SLRPs, DSPG3 is predominantly expressed in cartilage. In this study, we have determined that the human DSPG3 gene is composed of seven exons: Exon 2 of DSPG3 includes the start codon, exons 4-7 code for the leucine-rich repeats, exons 3 and 7 contain the potential glycosaminoglycan attachment sites, and exon 7 contains the potential N-glycosylation sites and the stop codon. We have identified two polymorphic variations, an insertion/deletion composed of 19 nucleotides in intron 1 and a tetranucleotide (TATT)n repeat in intron 5. Analysis of 1.6 kb of upstream promoter sequence of DSPG3 reveals three TATA boxes, one of which is 20 nucleotides before the transcription start site. The transcription start site precedes the translation start site by 98 nucleotides. There are 14 potential binding sites for SOX9, a transcription factor present in cartilage, in the promoter, and in the first intron of DSPG3. We have examined the evolution of the SLRP gene family and found that gene products clustered together in the evolutionary tree are encoded by genes with similarities in genomic structure. Hence, it appears that the majority of the introns in the SLRP genes were inserted after the differentiation of the SLRP genes from an ancestral gene that was most likely composed of 2-3 exons.
DSPG3是鸡PG-Lb的人类同源物,是富含亮氨酸的小分子重复蛋白聚糖(SLRP)家族的成员之一,该家族还包括核心蛋白聚糖、双糖链蛋白聚糖、纤维调节蛋白和纤连蛋白。与其他SLRP的组织分布不同,DSPG3主要在软骨中表达。在本研究中,我们确定人类DSPG3基因由七个外显子组成:DSPG3的外显子2包含起始密码子,外显子4至7编码富含亮氨酸的重复序列,外显子3和7含有潜在的糖胺聚糖附着位点,外显子7含有潜在的N-糖基化位点和终止密码子。我们鉴定出两个多态性变异,一个是内含子1中由19个核苷酸组成的插入/缺失,另一个是内含子5中的四核苷酸(TATT)n重复序列。对DSPG3上游1.6 kb启动子序列的分析揭示了三个TATA框,其中一个位于转录起始位点前20个核苷酸处。转录起始位点比翻译起始位点靠前98个核苷酸。在DSPG3的启动子和第一个内含子中有14个潜在的SOX9结合位点,SOX9是一种存在于软骨中的转录因子。我们研究了SLRP基因家族的进化,发现进化树中聚集在一起的基因产物由基因组结构相似的基因编码。因此,似乎SLRP基因中的大多数内含子是在SLRP基因从一个最可能由2-3个外显子组成的祖先基因分化后插入的。