Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

Dayle McClintock; Leslie B. Gordon; Karima Djabali

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Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

机译：Hutchinson-Gilford早衰突变体Lamin A主要通过抗Lamin A G608G抗体检测到靶向人血管细胞

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Hutchinson-Gilford progeria syndrome (HGPS; Online Mendelian Inheritance in Man accession no. 176670) is a rare disorder that is characterized by segmental premature aging and death between 7 and 20 years of age from severe premature atherosclerosis. Mutations in the LMNA gene are responsible for this syndrome. Approximately 80% of HGPS cases are caused by a G608 (GGC→GGT) mutation within exon 11 of LMNA, which elicits a deletion of 50 aa near the C terminus of prelamin A. In this article, we present evidence that the mutant lamin A (progerin) accumulates in the nucleus in a cellular age-dependent manner. In human HGPS fibroblast cultures, we observed, concomitantly to nuclear progerin accumulation, severe nuclear envelope deformations and invaginations preventable by farnesyltransferase inhibition. Nuclear alterations affect cell-cycle progression and cell migration and elicit premature senescence. Strikingly, skin biopsy sections from a subject with HGPS showed that the truncated lamin A accumulates primarily in the nuclei of vascular cells. This finding suggests that accumulation of progerin is directly involved in vascular disease in progeria.

机译：Hutchinson-Gilford早衰综合症（HGPS；在线孟德尔遗传在线登录号176670）是一种罕见疾病，其特征是在严重的早发性动脉粥样硬化中出现节段性早衰并在7至20岁之间死亡。 LMNA基因突变是导致这种综合征的原因。大约80％的HGPS病例是由LMNA外显子11内的G608（GGC→GGT）突变引起的，该突变在前乳蛋白A的C末端附近缺失了50个氨基酸。在本文中，我们提供了证据证明突变型层粘蛋白A （progerin）以细胞年龄依赖性方式积累在细胞核中。在人类HGPS成纤维细胞培养物中，我们观察到核早老蛋白积累，严重的核被膜变形和法尼基转移酶抑制可预防的内陷。核改变影响细胞周期进程和细胞迁移并引起过早衰老。令人惊讶的是，来自患有HGPS的受试者的皮肤活检切片显示，截短的核纤层蛋白A主要积聚在血管细胞核中。该发现表明，早衰素的积累直接参与早衰的血管疾病。

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来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2006年第7期|p.2154-2159|共6页
作者
Dayle McClintock; Leslie B. Gordon; Karima Djabali;
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作者单位

Department of Dermatology, College of Physicians and Surgeons, Columbia University, New York, NY 10032;

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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
中图分类自然科学总论;
关键词
aging; atherosclerosis;

机译：衰老;动脉粥样硬化;

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1. Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress [J] . Mauro Paradisi, Dayle McClintock, Revekka L Boguslavsky, BMC Cell Biology . 2005,第1期

机译：Hutchinson-Gilford早衰综合症的Lamin A G608G突变的皮肤成纤维细胞具有畸形核，对热应激过敏
2. Na???ˉve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo Na???ˉve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo Na???ˉve adult stem cells from patients with Hutchinson-Gilford progeria syndrome express low levels of progerin in vivo [J] . Leslie B. Gordon, Reinhard Schneider, Daniela Roedl, Biology Open . 2012,第6期

机译：患有Hutchinson-Gilford早衰综合症患者的幼体成年干细胞在体内表达低水平的早老素Hutchinson-Gilford早衰综合征患者的五个成体干细胞在体内表达的早孕素水平低
3. A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells [J] . Kan Cao, Brian C. Capell, Michael R. Erdos, Proceedings of the National Academy of Sciences of the United States of America . 2007,第12期

机译：在Hutchinson-Gilford早衰综合征中过表达的Lamin A蛋白同工型干扰早衰和正常细胞中的有丝分裂
4. CONTACTLESS INVESTIGATION OF NUCLEAR MECHANICS OF NORMAL AND LAMIN MUTANT CELLS USING A 3D IMAGE- AND MODEL-BASED FRAMEWORK [C] . Paula Gonzalez, Michaela Reichenzeller, Roland Eils, ASME summer bioengineering conference;SBC2009 . 2009

机译：使用基于3D图像和模型的框架对正常和层状突变细胞的核力学进行无接触研究
5. Hutchinson Gilford Progeria Syndrome and the role of lamin A in the onset of the progeroid cellular phenotype. [D] . Candelario, Jose. 2010

机译：哈钦森·吉尔福德（Hutchinson Gilford）早衰综合症和层粘连蛋白A在早发型细胞表型发作中的作用。
6. Hutchinson–Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody [O] . Dayle McClintock, Leslie B. Gordon, Karima Djabali 2006

机译：Hutchinson–Gilford早衰突变体Lamin A主要通过抗Lamin A G608G抗体检测到靶向人血管细胞
7. Hutchinson–Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody [O] . McClintock, Dayle, Gordon, Leslie B., Djabali, Karima 2006

机译：Hutchinson–Gilford早衰突变体Lamin A主要通过抗Lamin A G608G抗体检测到靶向人血管细胞
8. Simultaneous Vascular Targeting and Tumor Targeting of Cerebral Breast Cancer Metastases Using a T Cell Receptor Mimic Antibody. [R] . Bickel, U. 2015

机译：使用T细胞受体模拟抗体同时血管靶向和肿瘤靶向脑乳腺癌转移。

Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody

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