オルニチンカルバモイルトランスフェラーゼ
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- 1. 成人における肝性脳症:治療hepatic encephalopathy in adults treatment [show details]
…hyperammonemia. Ornithine serves both as an activator of carbamyl phosphate synthetase and ornithine-carbamyl transferase in periportal hepatocytes and as a substrate for ureagenesis. Ornithine (via alpha-ketoglutarate) …
- 2. 学会が公開する診療ガイドラインのリンク:尿素サイクル異常症society guideline links urea cycle disorders [show details]
… (2018) ACMG: Transition to adult health care ACT sheet – Carbamoyl phosphate synthetase I (CPS I) deficiency [urea cycle… ACMG: Transition to adult health care ACT sheet – Ornithine transcarbamylase (OTC) deficiency [urea cycle disorder]…
- 3. 尿素サイクル障害:管理urea cycle disorders management [show details]
…in the pathway causes a urea cycle disorder (UCD). The UCDs are: Carbamoyl phosphate synthetase I (CPSI) deficiency (MIM #237300) Ornithine transcarbamylase (OTC) deficiency (MIM #311250) Argininosuccinate …
- 4. 肝細胞移植hepatocyte transplantation [show details]
… in culture have been transplanted in a child with ornithine carbamoyltransferase deficiency .… ammonia concentration and improved urea production for up to 11 months in a 2.5-month-old child with carbamoyl phosphate synthetase I deficiency, after transplantation of 1.4 × 109 cryopreserved hepatocytes…
- 5. 小児に急性発症する精神病の救急外来での評価emergency department evaluation of acute onset psychosis in children [show details]
… This delayed presentation is most common with partial ornithine transcarbamylase deficiency,… aspartate transferase [AST], alanine transferase [ALT]) and liver function tests (total and direct bilirubin,…
English Journal
- [Genetic testing and prenatal diagnosis in seven pedigrees affected with ornithine transcarbamylase deficiency].
- Liu N, Feng Y, Jiang M, Kong X.
- Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics. 2020 Feb;37(2)106-109.
- To detect genetic variants among 7 pedigrees affected with ornithine transcarbamylase deficiency (OTCD) and provide prenatal diagnosis for them. The pedigrees were subjected to targeted next-generation sequencing (NGS) with a gene panel for inherited metabolic diseases. Suspected pathological varian
- PMID 32034732
- Probing remote residues important for catalysis in Escherichia coli ornithine transcarbamoylase.
- Ngu L, Winters JN, Nguyen K, Ramos KE, DeLateur NA, Makowski L, Whitford PC, Ondrechen MJ, Beuning PJ.
- PloS one. 2020 ;15(2)e0228487.
- Understanding how enzymes achieve their tremendous catalytic power is a major question in biochemistry. Greater understanding is also needed for enzyme engineering applications. In many cases, enzyme efficiency and specificity depend on residues not in direct contact with the substrate, termed remot
- PMID 32027716
- Type I Interferon Signaling Disrupts the Hepatic Urea Cycle and Alters Systemic Metabolism to Suppress T Cell Function.
- Lercher A, Bhattacharya A, Popa AM, Caldera M, Schlapansky MF, Baazim H, Agerer B, Gürtl B, Kosack L, Májek P, Brunner JS, Vitko D, Pinter T, Genger JW, Orlova A, Pikor N, Reil D, Ozsvár-Kozma M, Kalinke U, Ludewig B, Moriggl R, Bennett KL, Menche J, Cheng PN, Schabbauer G, Trauner M, Klavins K, Bergthaler A.
- Immunity. 2019 12;51(6)1074-1087.e9.
- Infections induce complex host responses linked to antiviral defense, inflammation, and tissue damage and repair. We hypothesized that the liver, as a central metabolic hub, may orchestrate systemic metabolic changes during infection. We infected mice with chronic lymphocytic choriomeningitis virus
- PMID 31784108
Japanese Journal
- 酵素関係(アイソザイムを含む) オルニチンカルバモイルトランスフェラーゼ(OCT) (広範囲 血液・尿化学検査,免疫学的検査(第7版・1)その数値をどう読むか) -- (生化学的検査(1))
- セスバニア(Sesbania cannabina Pers. and Sesbania rostrata)サイレージの給与が育成牛の増体と血液性状に及ぼす影響
- 池田 健児,仮屋 喜弘,高野 俊彦 [他],永西 修,四十万谷 吉郎,村井 勝
- 日本草地学会誌 42(4), 353-359, 1997
- セスバニアの反別家畜の粗飼料としての適性や利用性を調べるため,二種類のセスバニアサイレージ(カンナビナおよびロストラータ)をホルスタイン種育成去勢牛におのおの3ヵ月間給与し,増体および血液性状に及ぼす影響を検討した。セスバニアサイレージの嗜好性については,個体によっては馴致するのに2週間程要するものもみられたが,それ以降の採食性は高かった。平均日増体量は両セスバニア区とも,対照のアルファルファ給与 …
- NAID 110006317994
- 土屋 亮 [他],藤瀬 浩,西薗 一也,芦田 佳典,山田 隆紹,小林 好作
- 日本獣医学雑誌 56(1), p21-26, 1994-02
- … 肝特異性の極めて高いことが知られているornithine carbamoyl transferase (OCT)活性の測定法を, 牛血清に適するように改良した. … 反応液中のcarbamoyl phosphate濃度を60mMに増量し, りん酸緩衝液をpH7.2にした. …
- NAID 110003915984
Related Links
- Ornithine transcarbamylase, acidurie orotique, transmission liée à l'X, hypocitrullinémie. Nom de la maladie et ses synonymes Déficit en ornithine-carbamyl-transferase Déficit en ornithine transcarbamylase (OTC) Le diagnostic
- ornithine ornithine ornithine ornithine ornithine acetyltransferase ornithine alpha-ketoglutarate Ornithine aminotransferase ornithine carbamoyl transferase (OCT) ornithine carbamoyl transferase (OCT), ornithine transcarbamoylase
- Ornithine carbamoyl transferase deficiency: findings, models and problems. Bachmann C(1). Author information: (1)Laboratoire Central de Chimie Clinique, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
★リンクテーブル★
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- 英
- ornithine carbamoyl transferase
- 同
- オルニチントランスカルバミラーゼ ornithine transcarbamoylase
- 関
- オルニチン回路、オルニチントランスカルバミラーゼ欠損症、
- カルバモイルリン酸+オルニチン--(オルニチンカルバモイルトランスフェラーゼ)-→シトルリン+リン酸
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- 関
- lyase、synthase、transposase
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トランスフェラーゼ