neuronal storage disease

神経元蓄積病

WordNet

1. an impairment of health or a condition of abnormal functioning
2. (computer science) the process of storing information in a computer memory or on a magnetic tape or disk
3. the act of storing something
4. the commercial enterprise of storing goods and materials
5. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. (倉庫などに)貯蔵すること,保管 / 貯蔵所,倉庫 / 保管料
4. 病気にかかった / 病的な,不健全な(morbid)
5. 神経単位,神経細胞,ニューロン,ノイロン

UpToDate Contents

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• 1. 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
• 2. ゴーシェ病：病因、臨床症状、および診断 gaucher disease pathogenesis clinical manifestations and diagnosis
• 3. 炎症性腸疾患における遺伝的要因 genetic factors in inflammatory bowel disease
• 4. ニーマン・ピック病の概要 overview of niemann pick disease
• 5. アレキサンダー病 alexander disease

English Journal

• The Chihuahua dog: A new animal model for neuronal ceroid lipofuscinosis CLN7 disease?

• Faller KM1, Bras J2, Sharpe SJ1, Anderson GW3, Darwent L2, Kun-Rodrigues C2, Alroy J4, Penderis J5, Mole SE6, Gutierrez-Quintana R1, Guerreiro RJ2.
• Journal of neuroscience research.J Neurosci Res.2016 Apr;94(4):339-47. doi: 10.1002/jnr.23710. Epub 2016 Jan 13.
• Neuronal ceroid lipofuscinoses (NCLs) are a group of incurable lysosomal storage disorders characterized by neurodegeneration and accumulation of lipopigments mainly within the neurons. We studied two littermate Chihuahua dogs presenting with progressive signs of blindness, ataxia, pacing, and cogni
• PMID 26762174

• Stimulation of adenosine A2A receptors reduces intracellular cholesterol accumulation and rescues mitochondrial abnormalities in human neural cell models of Niemann-Pick C1.

• Ferrante A1, De Nuccio C2, Pepponi R3, Visentin S2, Martire A3, Bernardo A2, Minghetti L2, Popoli P3.
• Neuropharmacology.Neuropharmacology.2016 Apr;103:155-62. doi: 10.1016/j.neuropharm.2015.11.022. Epub 2015 Nov 26.
• Niemann Pick C 1 (NPC1) disease is an incurable, devastating lysosomal-lipid storage disorder characterized by hepatosplenomegaly, progressive neurological impairment and early death. Current treatments are very limited and the research of new therapeutic targets is thus mandatory. We recently showe
• PMID 26631535

• A peptide-linked recombinant glucocerebrosidase for targeted neuronal delivery: Design, production, and assessment.

• Gramlich PA1, Westbroek W2, Feldman RA3, Awad O3, Mello N4, Remington MP5, Sun Y6, Zhang W7, Sidransky E2, Betenbaugh MJ8, Fishman PS9.
• Journal of biotechnology.J Biotechnol.2016 Mar 10;221:1-12. doi: 10.1016/j.jbiotec.2016.01.015. Epub 2016 Jan 18.
• Although recombinant glucocerebrosidase (GCase) is the standard therapy for the inherited lysosomal storage disease Gaucher's disease (GD), enzyme replacement is not effective when the central nervous system is affected. We created a series of recombinant genes/proteins where GCase was linked to dif
• PMID 26795355

Japanese Journal

• アルコール依存症とWernicke's encephalopathy(<特集>ビタミンB_1による認知症の予防・治療)

• 松井 敏史,櫻井 秀樹,遠山 朋海 [他],吉村 淳,松下 幸生,樋口 進
• ビタミン 86(11), 630-635, 2012-11-25
• … The plausible causes of B_1 deficiency in alcoholics depend on the underlying mechanisms, such as a low dietary intake of B_1, inadequate absorption of B_1 from the intestine due to the gastrointestinal tract damage, and coexistent alcoholic liver disease altering the capacity of B_1 storage and the metabolism of biologically important nutrients. …
• NAID 110009554187

• Cerebrospinal fluid biomarkers showing neurodegeneration in dogs with GM1 gangliosidosis : possible use for assessment of a therapeutic regimen

• Satoh Hiroyuki,Yamato Osamu,Asano Tomoya,Yonemura Madoka,Yamauchi Toyofumi,Hasegawa Daisuke,Orima Hiromitsu,Arai Toshiro,Yamasaki Masahiro,Maede Yoshimitsu
• Brain Research 1133(1), 200-208, 2007-02-16
• … GM1 gangliosidosis, a lysosomal storage disease that affects the brain and multiple systemic organs, is due to an autosomal recessively inherited deficiency of acid β-galactosidase activity. … Pathogenesis of GM1 gangliosidosis may include neuronal apoptosis and abnormal axoplasmic transport and inflammatory response, which are perhaps consequent to massive neuronal storage of GM1 ganglioside. …
• NAID 120000965135

• Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse

• LIU Y
• Hum Mol Genet 9, 1087-1092, 2000
• NAID 30018401630

Related Links

• Pathobiology of Neuronal Storage Disease - Steven Walkley

NIH 1994 R01 NS Pathobiology of Neuronal Storage Disease Walkley, Steven Upshaw / Albert Einstein College of Medicine NIH 1993 R01 NS Pathobiology of Neuronal Storage Disease Walkley, Steven Upshaw / Albert Einstein ...

• Neuronal storage disease in a group of captive Humboldt ...

1. Vet Pathol. 2006 Nov;43(6):1029-33. Neuronal storage disease in a group of captive Humboldt penguins (Spheniscus humboldti). Wünschmann A, Armien A, Wallace R, Wictor M, Oglesbee M. Department of Veterinary Population ...

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★リンクテーブル★

リンク元	「神経元蓄積病」
関連記事	「disease」「neuronal」「storage」

「神経元蓄積病」

　　[★]

英

neuronal storage disease

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「neuronal」

　　[★]

• 神経細胞の、ニューロンの、神経型の

関

nerve cell、neural、neural cell、neuron、neuronal cell、neurone

「storage」

　　[★]

• n.

• 貯蔵

関

pool、pooling、preservation、preserve、reserve、store