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交替表达的内皮细胞糖蛋白 Ibβ（GPIbβ）的互补 DNA 克隆及 GPIbβ 基因在 22 号染色体上的定位。

Complementary DNA cloning of the alternatively expressed endothelial cell glycoprotein Ib beta (GPIb beta) and localization of the GPIb beta gene to chromosome 22.

作者信息

Kelly M D, Essex D W, Shapiro S S, Meloni F J, Druck T, Huebner K, Konkle B A

机构信息

Cardeza Foundation for Hematologic Research, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania 19107.

出版信息

J Clin Invest. 1994 Jun;93(6):2417-24. doi: 10.1172/JCI117249.

DOI:10.1172/JCI117249
PMID:8200976
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC294449/
Abstract

Glycoprotein Ib beta (GPIb beta) exists in platelets disulfide-linked to glycoprotein Ib alpha (GPIb alpha), a major receptor for von Willebrand factor. Both GPIb alpha and GPIb beta are expressed in endothelial cells (EC). While the GPIb alpha mRNA and protein appear similar in platelets and EC, EC GPIb beta mRNA is larger than platelet GPIb beta and encodes a larger protein. We have cloned and sequenced EC GPIb beta cDNA and report a 2793-nucleotide sequence which contains a 411-amino acid open reading frame. The EC sequence contains all of the platelet cDNA sequence and all but three amino acids of the primary translation product. Like the genes encoding GPIb alpha, GPIX, and GPV, the GPIb beta gene appears simple in structure. Using human hamster hybrids, we have localized the GPIb beta gene to chromosome 22pter-->22q11.2. When we examined poly (A)+ RNA from several human tissues for GPIb beta mRNA expression, we found that GPIb beta mRNA was expressed in a variety of tissues but was most abundant in heart and brain, while GPIb alpha and GPIX mRNA expression was found only in lung and placenta at very low levels. The broad distribution of GPIb beta mRNA suggests that it may be playing a role different than or additional to its function in platelets.

摘要

糖蛋白 Ibβ（GPIbβ）存在于血小板中，通过二硫键与糖蛋白 Ibα（GPIbα）相连，GPIbα是血管性血友病因子的主要受体。GPIbα和GPIbβ均在内皮细胞（EC）中表达。虽然GPIbα的mRNA和蛋白质在血小板和EC中看起来相似，但EC的GPIbβmRNA比血小板的GPIbβmRNA更大，且编码的蛋白质也更大。我们已克隆并测序了EC的GPIbβcDNA，并报告了一个2793个核苷酸的序列，该序列包含一个411个氨基酸的开放阅读框。EC序列包含了所有血小板cDNA序列以及初级翻译产物中除三个氨基酸外的所有氨基酸。与编码GPIbα、GPIX和GPV的基因一样，GPIbβ基因的结构似乎很简单。利用人-仓鼠杂种细胞，我们已将GPIbβ基因定位到染色体22pter→22q11.2。当我们检测来自几种人类组织的多聚腺苷酸（poly(A)）+RNA中GPIbβmRNA的表达时，我们发现GPIbβmRNA在多种组织中表达，但在心脏和大脑中最为丰富，而GPIbα和GPIX mRNA仅在肺和胎盘中以极低水平表达。GPIbβmRNA的广泛分布表明，它可能在发挥与其在血小板中的功能不同或额外的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/221483489cc6/jcinvest00035-0130-f.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/150406419b2c/jcinvest00035-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/fb701f030425/jcinvest00035-0127-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/1e2708bbb623/jcinvest00035-0127-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/24da9b42c715/jcinvest00035-0127-d.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/17f4bfad4df1/jcinvest00035-0130-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/bb27a8121aeb/jcinvest00035-0130-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/6626a564c7f1/jcinvest00035-0130-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/db7fca3edf2f/jcinvest00035-0130-d.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/590ffd2617a0/jcinvest00035-0130-e.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/221483489cc6/jcinvest00035-0130-f.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/150406419b2c/jcinvest00035-0127-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/fb701f030425/jcinvest00035-0127-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/1e2708bbb623/jcinvest00035-0127-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/24da9b42c715/jcinvest00035-0127-d.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/17f4bfad4df1/jcinvest00035-0130-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/bb27a8121aeb/jcinvest00035-0130-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/6626a564c7f1/jcinvest00035-0130-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/db7fca3edf2f/jcinvest00035-0130-d.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/590ffd2617a0/jcinvest00035-0130-e.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/337c/294449/221483489cc6/jcinvest00035-0130-f.jpg

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