peroxisomal biogenesis disorders

同

PBD

WordNet

1. bring disorder to (同)disarray
2. a physical condition in which there is a disturbance of normal functioning; "the doctor prescribed some medicine for the disorder"; "everyone gets stomach upsets from time to time" (同)upset
3. a disturbance of the peace or of public order
4. the production of living organisms from other living organisms (同)biogeny

PrepTutorEJDIC

1. 〈U〉『無秩序』,混乱,乱雑(confusion) / 《しばしば複数形で》(社会的・政治的な)粉争,騒動 / 〈C〉(肉体的・精神的な)不調,異常,障害 / …‘の'秩序を乱す / 〈心身〉‘に'異常を起こさせる

UpToDate Contents

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• 1. ペルオキシソーム病 peroxisomal disorders
• 2. 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
• 3. 新生児における筋緊張低下を引き起こす末梢神経および筋肉の障害に関する概要 overview of peripheral nerve and muscle disorders causing hypotonia in the newborn
• 4. 先天性代謝異常：分類 inborn errors of metabolism classification
• 5. 全身疾患と関連した遺伝性ニューロパチー hereditary neuropathies associated with generalized disorders

English Journal

• SUMO-regulated mitochondrial function in Parkinson's disease.

• Guerra de Souza AC1, Prediger RD1, Cimarosti H1.
• Journal of neurochemistry.J Neurochem.2016 Jun;137(5):673-86. doi: 10.1111/jnc.13599. Epub 2016 Apr 5.
• Parkinson's disease (PD) is the second most common neurodegenerative disorder characterized by cardinal motor signs such as rigidity, bradykinesia or rest tremor that arise from a significant death of dopaminergic neurons. Non-dopaminergic degeneration also occurs and it seems to induce the deficits
• PMID 26932327

• Effect of l-Arginine in One Patient with Peroxisome Biogenesis Disorder due to PEX12 Deficiency.

• Sorlin A1, Briand G2, Cheillan D3, Wiedemann A1, Montaut-Verient B4, Schmitt E5, Feillet F1.
• Neuropediatrics.Neuropediatrics.2016 Jun;47(3):179-81. doi: 10.1055/s-0036-1578798. Epub 2016 Mar 4.
• Peroxisome biogenesis disorders (PBD) are a heterogeneous group of disorders due to PEX genes mutations, with a broad clinical spectrum comprising severe neonatal disease to mild presentation. Recently, Berendse et al reported an improvement of peroxisomal functions with l-arginine supplementation i
• PMID 26947510

• Living-Donor Liver Transplantation From a Heterozygous Parent for Infantile Refsum Disease.

• Matsunami M1, Shimozawa N2, Fukuda A3, Kumagai T4, Kubota M4, Chong PF5, Kasahara M3.
• Pediatrics.Pediatrics.2016 Jun;137(6). pii: e20153102. doi: 10.1542/peds.2015-3102.
• Infantile Refsum disease (IRD) is a rare autosomal recessive disorder of peroxisome biogenesis characterized by generalized peroxisomal metabolic dysfunction, including accumulation of very long-chain fatty acids (VLCFAs) and phytanic acid (PA), as well as decreased plasmalogen contents (PL). An eff
• PMID 27221287

Japanese Journal

• Transcriptome Analysis of K-877 (a Novel Selective PPARα Modulator (SPPARMα))-Regulated Genes in Primary Human Hepatocytes and the Mouse Liver

• Journal of Atherosclerosis and Thrombosis 22(8), 754-772, 2015
• NAID 130005095007

• Transcriptome Analysis of K-877 (a Novel Selective PPARα Modulator (SPPARMα))-Regulated Genes in Primary Human Hepatocytes and the Mouse Liver

• Journal of Atherosclerosis and Thrombosis advpub(0), 2015
• NAID 130005074459

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

• PLOS ONE 6(8), 2011-08
• NAID 120004996597

Related Links

• Peroxisome Biogenesis Disorders, Zellweger Syndrome ...

Testing Biochemical testing. Biochemical assays can determine definitively whether an individual has a peroxisomal biogenesis disorder, Zellweger syndrome spectrum (PBD, ZSS). The battery of biochemical analyses ...

• PEROXISOMAL DISORDERS - Neuropathology

PEROXISOMAL DISORDERS Peroxisomes are oxidative organelles that are present in all tissues but are especially abundant in the liver and kidney. As their name indicates, they use molecular oxygen in oxidative reactions that generate hydrogen peroxide

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★リンクテーブル★

リンク元	「PBD」
関連記事	「disorder」「biogenesis」「peroxisomal」

「PBD」

　　[★]

同

peroxisomal biogenesis disorders

「disorder」

　　[★]

• 障害：個人的苦痛や機能の障害があるので「疾病」とは言えるものの、その背景にある臓器障害がもう一つはっきりしない場合に用いられる。(PSY.9)

• n.

• an untidy state; a lack of order or organization (⇔order)
• violent behaviour of large groups of people
• an illness that cause a part of the body to stop functioning correctly

• 注意

disease <> illness <> disorder

• vt.

• 乱す、乱雑にする。(人)の(心身の)調子を狂わせる。

• vi.

　 　 　 　

「biogenesis」

　　[★]

生合成、バイオジェネシス

関

biogenetic、biosyntheses、biosynthesis、biosynthetic、origin of life

　

「peroxisomal」

　　[★]

• ペルオキシソームの

関

peroxisome