PAI-1 deficiency

プラスミノゲンアクチベーターインヒビター1欠乏症

WordNet

1. the 16th letter of the Roman alphabet (同)p

PrepTutorEJDIC

1. 〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
2. personal assistant個人秘書 / public address [system]
3. parking
4. phosphorusの化学記号

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• 1. 血栓溶解異常による血栓性および出血性疾患 thrombotic and hemorrhagic disorders due to abnormal fibrinolysis
• 2. 稀な遺伝性凝固異常 rare inherited coagulation disorders
• 3. 出血性素因を有する成人患者へのアプローチ approach to the adult patient with a bleeding diathesis
• 4. 補体第1成分阻害因子に遺伝子異常のない遺伝性血管性浮腫 hereditary angioedema with normal c1 inhibitor
• 5. 皮斑様血管障害 livedoid vasculopathy

English Journal

• Plasminogen activator inhibitor-1 deficiency ameliorates insulin resistance and hyperlipidemia but not bone loss in obese female mice.

• Tamura Y1, Kawao N, Yano M, Okada K, Matsuo O, Kaji H.Author information 1Department of Physiology and Regenerative Medicine (Y.T., N.K., M.Y., K.O., H.K.), Kinki University Faculty of Medicine (O.M.), Osakasayama 589-8511, Japan.AbstractWe previously demonstrated that plasminogen activator inhibitor-1 (PAI-1), an inhibitor of fibrinolysis, is involved in type 1 diabetic bone loss in female mice. PAI-1 is well known as an adipogenic factor induced by obesity. We therefore examined the effects of PAI-1 deficiency on bone and glucose and lipid metabolism in high-fat and high-sucrose diet (HF/HSD)-induced obese female mice. Female wild-type (WT) and PAI-1-deficient mice were fed with HF/HSD or normal diet for 20 weeks from 10 weeks of age. HF/HSD increased the levels of plasma PAI-1 in WT mice. PAI-1 deficiency suppressed the levels of blood glucose, plasma insulin, and total cholesterol elevated by obesity. Moreover, PAI-1 deficiency improved glucose intolerance and insulin resistance induced by obesity. Bone mineral density (BMD) at trabecular bone as well as the levels of osterix, alkaline phosphatase, and receptor activator of nuclear factor κB ligand mRNA in tibia were decreased by HF/HSD in WT mice, and those changes by HF/HSD were not affected by PAI-1 deficiency. HF/HSD increased the levels of plasma TNF-α in both WT and PAI-1-deficient mice, and the levels of plasma TNF-α were negatively correlated with trabecular BMD in tibia of female mice. In conclusion, we revealed that PAI-1 deficiency does not affect the trabecular bone loss induced by obesity despite the amelioration of insulin resistance and hyperlipidemia in female mice. Our data suggest that the changes of BMD and bone metabolism by obesity might be independent of PAI-1 as well as glucose and lipid metabolism.
• Endocrinology.Endocrinology.2014 May;155(5):1708-17. doi: 10.1210/en.2013-1888. Epub 2014 Feb 28.
• We previously demonstrated that plasminogen activator inhibitor-1 (PAI-1), an inhibitor of fibrinolysis, is involved in type 1 diabetic bone loss in female mice. PAI-1 is well known as an adipogenic factor induced by obesity. We therefore examined the effects of PAI-1 deficiency on bone and glucose
• PMID 24605827

• The obstetric, gynaecological and fertility implications of homozygous PAI-1 deficiency: single-centre experience.

• Heiman M1, Gupta S, Shapiro AD.Author information 1Indiana Hemophilia and Thrombosis Center, Indianapolis, IN, USA.AbstractComplete plasminogen activator inhibitor type 1 (PAI-1) deficiency is an exceedingly rare autosomal recessive bleeding disorder previously identified and reported in a large Old Order Amish (OOA) kindred in Indiana [Fay et al. Blood 1997; 90: 204]. Mouse models suggest that proteolysis via the plasminogen activator/plasmin system plays a crucial role in reproduction including degradation of the follicular wall during ovulation, fertilization, embryo implantation and embryogenesis [Leonardsson et al., Proc Natl Acad Sci USA 1995; 92: 12446]. We report the obstetric, gynaecological and fertility histories of OOA individuals with homozygous PAI-1 deficiency. In this family, there are 10 affected members identified to date ranging in age between 10 and 32 years, including seven female patients and three male patients. To date, two women have achieved pregnancies without difficulty; however, they experienced antenatal bleeding and preterm labour. The early initiation and continuation of antifibrinolytic agents, Epsilon-aminocaproic acid or tranexamic acid, during the pregnancy and in the postpartum period, was believed to be successful in preventing major bleeding complications in our patients with complete PAI-1 deficiency.
• Haemophilia : the official journal of the World Federation of Hemophilia.Haemophilia.2014 May;20(3):407-12. doi: 10.1111/hae.12313. Epub 2013 Nov 22.
• Complete plasminogen activator inhibitor type 1 (PAI-1) deficiency is an exceedingly rare autosomal recessive bleeding disorder previously identified and reported in a large Old Order Amish (OOA) kindred in Indiana [Fay et al. Blood 1997; 90: 204]. Mouse models suggest that proteolysis via the plas
• PMID 24261743

• PAI-1-regulated extracellular proteolysis governs senescence and survival in Klotho mice.

• Eren M1, Boe AE, Murphy SB, Place AT, Nagpal V, Morales-Nebreda L, Urich D, Quaggin SE, Budinger GR, Mutlu GM, Miyata T, Vaughan DE.Author information 1Department of Medicine and Feinberg Cardiovascular Research Institute, Northwestern University Feinberg School of Medicine, Chicago, IL 60611.AbstractCellular senescence restricts the proliferative capacity of cells and is accompanied by the production of several proteins, collectively termed the "senescence-messaging secretome" (SMS). As senescent cells accumulate in tissue, local effects of the SMS have been hypothesized to disrupt tissue regenerative capacity. Klotho functions as an aging-suppressor gene, and Klotho-deficient (kl/kl) mice exhibit an accelerated aging-like phenotype that includes a truncated lifespan, arteriosclerosis, and emphysema. Because plasminogen activator inhibitor-1 (PAI-1), a serine protease inhibitor (SERPIN), is elevated in kl/kl mice and is a critical determinant of replicative senescence in vitro, we hypothesized that a reduction in extracellular proteolytic activity contributes to the accelerated aging-like phenotype of kl/kl mice. Here we show that PAI-1 deficiency retards the development of senescence and protects organ structure and function while prolonging the lifespan of kl/kl mice. These findings indicate that a SERPIN-regulated cell-nonautonomous proteolytic cascade is a critical determinant of senescence in vivo.
• Proceedings of the National Academy of Sciences of the United States of America.Proc Natl Acad Sci U S A.2014 Apr 28. [Epub ahead of print]
• Cellular senescence restricts the proliferative capacity of cells and is accompanied by the production of several proteins, collectively termed the "senescence-messaging secretome" (SMS). As senescent cells accumulate in tissue, local effects of the SMS have been hypothesized to disrupt tissue regen
• PMID 24778222

Japanese Journal

• ヒトとマウスにおけるPAI-1欠損の表現型の違い

• 梅村 和夫
• 日本血栓止血学会誌 24(5), 520-522, 2013
• NAID 130003384148

• PAI-1 欠損症患者との出会い

• 小林 隆夫
• 日本血栓止血学会誌 24(4), 460-462, 2013
• NAID 130003367372

• 一酸化炭素(CO)供与体由来COがヒト血管内皮細胞での組織因子およびプラスミノーゲンアクチバータータイプ1を調節する

• Maruyama Keiko,Morishita Eriko,Yuno Takeo,Sekiya Akiko,Asakura Hidesaku,Ohtake Shigeki,Yachie Akihiro
• Thrombosis Research 130(3), e188-e193, 2012-09
• … Introduction: Heme oxygenase-1 (HO-1) is the rate limiting enzyme that catalyzes the conversion of heme into biliverdin, free iron, and carbon monoxide (CO). … The first human case of HO-1 deficiency showed abnormalities in blood coagulation and the fibrinolytic system. … Thus, HO-1 or HO-1 products, such as CO, might regulate coagulation and the fibrinolytic system. …
• NAID 120005285926

Related Links

• Plasminogen activator inhibitor type 1 deficiency.

1. Haemophilia. 2008 Nov;14(6):1255-60. doi: 10.1111/j.1365-2516.2008.01834.x. Plasminogen activator inhibitor type 1 deficiency. Mehta R(1), Shapiro AD. Author information: (1)Department of Clinical Medicine, Section of ...

• Plasminogen Activator Inhibitor Type 1 Deficiency

Plasminogen activator inhibitor-1 (PAI-1) deficiency is a rare inherited autosomal recessive bleeding disorder. ... Background Epidemiology Plasminogen activator inhibitor-1 (PAI-1) deficiency is a rare inherited autosomal recessive ...

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★リンクテーブル★

リンク元	「プラスミノゲンアクチベーターインヒビター1欠乏症」
関連記事	「deficiency」「PA」「P」「PAI」「pai」

「プラスミノゲンアクチベーターインヒビター1欠乏症」

　　[★]

英

plasminogen activator inhibitor 1 deficiency

同

PAI-1 deficiency

関

プラスミノゲン、プラスミノーゲンアクチベーターインヒビター1 PAI-1、プラスミノーゲンアクチベーター、プラスミノーゲンアクチベーター

[show details]

プラスミノーゲンアクチベーターインヒビター1欠乏症 : nothing
プラスミノゲンアクチベーターインヒビター1欠乏症 : 1 件

「deficiency」

　　[★]

• n.

• 不足、欠乏、欠失、欠如、欠損、不十分。栄養不足、栄養素欠乏、欠乏症。(遺伝子)(染色体内の)遺伝子欠失
• 欠けているもの、不足している物。不足分。不完全なもの、欠点のあるもの

関

absence, agenesis, dearth, defect, defective, deficient, deficit, delete, deletion, deletional, depletion, deprivation, deprive, lack, miss, missing, morphological defect, paucity, scarce, scarcity, starve

　

「PA」

　　[★]

• 肺動脈弁閉鎖症 pulmonary atresia
• 肺動脈 pulmonary artery、肺動脈幹
• 原発性アルドステロン症 primary aldosteronism

• 微生物学：粒子凝集法(particle agglutination test)
• 薬理学：

　

「P」

　　[★]

• プロリン proline
• P波 P wave
• リン phosphorus

　 　 　

「PAI」

　　[★] プラスミノーゲンアクチベーターインヒビター

「pai」

　　[★]

• n.

• (ギリシャ文字)π、パイ