multiple peroxisomal dysfunction

多発性ペルオキシソーム障害

関

hyperpipecolic acidemia、neonatal adrenoleukodystrophy、peroxisomal disorder

WordNet

1. (medicine) any disturbance in the functioning of an organ or body part or a disturbance in the functioning of a social group; "erectile dysfunction"; "sexual relationship dysfunction" (同)disfunction
2. having or involving or consisting of more than one part or entity or individual; "multiple birth"; "multiple ownership"; "made multiple copies of the speech"; "his multiple achievements in public life"; "her multiple personalities"; "a pineapple is a multiple fruit"
3. the product of a quantity by an integer; "36 is a multiple of 9"

PrepTutorEJDIC

1. 機能障害,機能不全
2. 多数の部分(要素)から成る,複合の,複式の / 倍数

UpToDate Contents

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• 1. 虚血後（虚血性）の急性尿細管壊死の発症および病因 pathogenesis and etiology of postischemic ischemic acute tubular necrosis
• 2. 虚血後（虚血性）の急性尿細管壊死において考え得る予防および治療 possible prevention and therapy of postischemic ischemic acute tubular necrosis
• 3. ペルオキシソーム病 peroxisomal disorders
• 4. 敗血症および全身性炎症反応症候群：定義、疫学、および予後 sepsis and the systemic inflammatory response syndrome definitions epidemiology and prognosis
• 5. 小児における原発性副腎不全の原因および臨床症状 causes and clinical manifestations of primary adrenal insufficiency in children

English Journal

• Peroxisomal lipid synthesis regulates inflammation by sustaining neutrophil membrane phospholipid composition and viability.

• Lodhi IJ1, Wei X1, Yin L1, Feng C1, Adak S1, Abou-Ezzi G2, Hsu FF1, Link DC2, Semenkovich CF3.
• Cell metabolism.Cell Metab.2015 Jan 6;21(1):51-64. doi: 10.1016/j.cmet.2014.12.002.
• Fatty acid synthase (FAS) is altered in metabolic disorders and cancer. Conventional FAS null mice die in utero, so effects of whole-body inhibition of lipogenesis following development are unknown. Inducible global knockout of FAS (iFASKO) in mice was lethal due to a disrupted intestinal barrier an
• PMID 25565205

• Proteasome inhibitors induce auditory hair cell death through peroxisome dysfunction.

• Lee JN1, Kim SG1, Lim JY1, Kim SJ1, Choe SK1, Park R2.
• Biochemical and biophysical research communications.Biochem Biophys Res Commun.2015 Jan 2;456(1):269-74. doi: 10.1016/j.bbrc.2014.11.070. Epub 2014 Nov 25.
• Even though bortezomib, a proteasome inhibitor, is a powerful chemotherapeutic agent used to treat multiple myeloma (MM) and other lymphoma cells, recent clinical reports suggest that the proteasome inhibitor therapy may be associated with severe bilateral hearing loss. We herein investigated the ad
• PMID 25446082

• An overview of inborn errors of complex lipid biosynthesis and remodelling.

• Lamari F1, Mochel F, Saudubray JM.
• Journal of inherited metabolic disease.J Inherit Metab Dis.2015 Jan;38(1):3-18. doi: 10.1007/s10545-014-9764-x. Epub 2014 Sep 20.
• In a review published in 2012, we delineated 14 inborn errors of metabolism (IEM) related to defects in biosynthesis of complex lipids, particularly phospholipids and sphingolipids (Lamari et al 2013). Given the numerous roles played by these molecules in membrane integrity, cell structure and funct
• PMID 25238787

Japanese Journal

• Drosophila Carrying Pex3 or Pex16 Mutations Are Models of Zellweger Syndrome That Reflect Its Symptoms Associated with the Absence of Peroxisomes

• Nakayama Minoru,Sato Hiroyasu,Okuda Takayuki,Fujisawa Nao,Kono Nozomu,Arai Hiroyuki,Suzuki Emiko,Umeda Masato,Ishikawa Hiroyuki O.,Matsuno Kenji
• PLOS ONE 6(8), 2011-08
• … The peroxisome biogenesis disorders (PBDs) are currently difficult-to-treat multiple-organ dysfunction disorders that result from the defective biogenesis of peroxisomes. … The human peroxin genes PEX3 or PEX16 are required for peroxisomal membrane protein targeting, and their mutations cause Zellweger syndrome, a class of PBDs. …
• NAID 120004996597

Related Links

• Peroxisomal Disorders

On the other hand, some doctors hold that since the peroxisome dysfunction is global and involves so many different abnormalities (in relations that aren't even fully known) the overall complex is beyond this sort of correction. Consistent ...

• Peroxisomal diseases

Most peroxisomal disorders cause severe neurological dysfunction due to CNS malformations (migration defects), myelin ... The unique biogenesis of peroxisomes and multiple interactions of PEX genes explain the genetic and phenotypic ...

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★リンクテーブル★

リンク元	「多発性ペルオキシソーム障害」「peroxisomal disorder」「hyperpipecolic acidemia」
関連記事	「dysfunction」「multiple」「peroxisomal」

「多発性ペルオキシソーム障害」

　　[★]

英

multiple peroxisomal dysfunction

関

ペルオキシソーム病、新生児副腎白質ジストロフィー、高ピペコリン酸血症

「peroxisomal disorder」

　　[★]

ペルオキシソーム病

関

hyperpipecolic acidemia、multiple peroxisomal dysfunction、neonatal adrenoleukodystrophy

「hyperpipecolic acidemia」

　　[★]

高ピペコリン酸血症

関

multiple peroxisomal dysfunction、neonatal adrenoleukodystrophy、peroxisomal disorder

「dysfunction」

　　[★]

• n.

• 機能不全、機能障害、機能異常

• vi

• 機能不全に陥る

関

barrier、damage、difficulty、disorder、disturbance、foe、functional disorder、functional impairment、hindrance、hypofunction、impair、impairment、impediment、incompetence、insufficiency、lesion、malfunction、obstacle、stun

　 　

「multiple」

　　[★]

• n.

• 倍数

• adj.

関

multifocality、multiplex、plural

　 　 　

「peroxisomal」

　　[★]

• ペルオキシソームの

関

peroxisome