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a white crystalline amino acid occurring in proteins that is essential for nutrition; obtained by the hydrolysis of most dietary proteins

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/11/19 04:18:41」(JST)

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1. グルコース-6-リン酸脱水素酵素欠損症の診断および治療 diagnosis and treatment of glucose 6 phosphate dehydrogenase deficiency
2. 乳酸脱水素酵素欠損症 lactate dehydrogenase deficiency
3. グルコース-6-リン酸脱水素酵素欠損症の臨床症状 clinical manifestations of glucose 6 phosphate dehydrogenase deficiency
4. 幼児の持続性高インスリン血症性低血糖症の病因、臨床的特徴、および診断 pathogenesis clinical features and diagnosis of persistent hyperinsulinemic hypoglycemia of infancy
5. グルコース-6-リン酸脱水素酵素欠損症の遺伝学および病態生理 genetics and pathophysiology of glucose 6 phosphate dehydrogenase deficiency

English Journal

A one-pot system for production of L-2-aminobutyric acid from L-threonine by L-threonine deaminase and a NADH-regeneration system based on L-leucine dehydrogenase and formate dehydrogenase.

Tao R1, Jiang Y, Zhu F, Yang S.Author information 1Key Laboratory of Synthetic Biology, Institute of Plant Physiology and Ecology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai, 200032, China, rstao@cibt.ac.cn.AbstractL-2-Aminobutyric acid (L-ABA) is an unnatural amino acid that is a key intermediate for the synthesis of several important drugs. It can be produced by transaminase or dehydrogenase from α-ketobutyric acid, which can be synthesized enzymatically from the bulk amino acid, L-threonine. Deamination of L-threonine followed by a hydrogenation reaction gave almost the theoretical yield and was estimated to be more cost-effective than the established chemical process. L-Threonine deaminase from Escherichia coli, L-leucine dehydrogenase from Bacillus cereus, and formate dehydrogenase from Pseudomonas sp. were over-expressed in E. coli and used for one-pot production of L-ABA with formate as a co-substrate for NADH regeneration. 30 mol L-threonine were converted to 29.2 mol L-ABA at 97.3 % of theoretical yield and with productivity of 6.37 g l(-1) h(-1) at 50 l. This process offers a promising approach to fulfil industrial requirements for L-ABA.
Biotechnology letters.Biotechnol Lett.2014 Apr;36(4):835-41. doi: 10.1007/s10529-013-1424-y. Epub 2013 Dec 10.
L-2-Aminobutyric acid (L-ABA) is an unnatural amino acid that is a key intermediate for the synthesis of several important drugs. It can be produced by transaminase or dehydrogenase from α-ketobutyric acid, which can be synthesized enzymatically from the bulk amino acid, L-threonine. Deamination of
PMID 24322776

Metabolism of activated T lymphocytes.

Maciolek JA1, Alex Pasternak J1, Wilson HL2.Author information 1Vaccine and Infectious Disease Organization (VIDO)-Home of the International Vaccine Centre (InterVac), University of Saskatchewan, 120 Veterinary Road, Saskatoon, S7N 5E3, Canada.2Vaccine and Infectious Disease Organization (VIDO)-Home of the International Vaccine Centre (InterVac), University of Saskatchewan, 120 Veterinary Road, Saskatoon, S7N 5E3, Canada. Electronic address: heather.wilson@usask.ca.AbstractActivated T cells undergo metabolic reprogramming which promotes glycolytic flux and lactate production as well as elevated production of lipids, proteins, nucleic acids and other carbohydrates (i.e. induction of biomass) even in the presence of oxygen. Activated T cells show induced expression of, among other things, Glucose Transporter 1 and several glycolytic enzymes, including ADP-Dependent Glucokinase and the low affinity isoform Pyruvate Kinase-M2 (which promote glycolytic flux), as well Glutamine Transporters and Glycerol-3-phosphate Dehydrogenase 2 which make available glutamate and glycerol-3-phosphate as mitochondrial energy sources. Intracellular leucine concentrations critically regulate mammalian target of rapamycin (mTOR) signaling to promote Th1, Th2, and Th17 CD4+ T effector cell differentiation. In contrast, T regulatory (Treg) cells are generated when AMP-Activating Protein Kinase signaling is activated and mTOR activation is suppressed. Unlike effector CD4+ and CD8+ T cells, Tregs and memory T cells oxidize fatty acids for fuel. Effector and memory T cells perform different functions and thus show distinct metabolic profiles which are exquisitely controlled by cellular signaling. Upon activation, T cells express the insulin and leptin receptors on their surface and become sensitive to insulin signaling and nutrient availability and show changes in differentiation. Thus, metabolism and nutrient availability influence T cell activation and function.
Current opinion in immunology.Curr Opin Immunol.2014 Apr;27C:60-74. doi: 10.1016/j.coi.2014.01.006. Epub 2014 Feb 18.
Activated T cells undergo metabolic reprogramming which promotes glycolytic flux and lactate production as well as elevated production of lipids, proteins, nucleic acids and other carbohydrates (i.e. induction of biomass) even in the presence of oxygen. Activated T cells show induced expression of,
PMID 24556090

Branched-chain amino acid metabolism: from rare Mendelian diseases to more common disorders.

Burrage LC1, Nagamani SC, Campeau PM, Lee BH.Author information 1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.AbstractBranched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the cause of maple syrup urine disease (MSUD) for decades, treatment options for this disorder have been limited to dietary interventions. In recent years, the discovery of improved leucine tolerance after liver transplantation has resulted in a new therapeutic strategy for this disorder. Likewise, targeting the regulation of the BCKDC activity may be an alternative potential treatment strategy for MSUD. The regulation of the BCKDC by the branched-chain ketoacid dehydrogenase kinase has also been implicated in a new inborn error of metabolism characterized by autism, intellectual disability and seizures. Finally, there is a growing body of literature implicating BCAA metabolism in more common disorders such as the metabolic syndrome, cancer and hepatic disease. This review surveys the knowledge acquired on the topic over the past 50 years and focuses on recent developments in the field of BCAA metabolism.
Human molecular genetics.Hum Mol Genet.2014 Apr 1. [Epub ahead of print]
Branched-chain amino acid (BCAA) metabolism plays a central role in the pathophysiology of both rare inborn errors of metabolism and the more common multifactorial diseases. Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their keto
PMID 24651065

Japanese Journal

Phenotypic Variability and Newly Identified Mutations of the IVD Gene in Japanese Patients with Isovaleric Acidemia

Sakamoto Osamu,Arai-Ichinoi Natsuko,Mitsubuchi Hiroshi,Chinen Yasutsugu,Haruna Hidenori,Maruyama Hidehiko,Sugawara Hidenori,Kure Shigeo
The Tohoku Journal of Experimental Medicine 236(2), 103-106, 2015
… Isovaleric acidemia (IVA) is an autosomal recessive inborn error affecting leucine metabolism. … It is caused by a deficiency in isovaleryl-CoA dehydrogenase (IVD), a mitochondrial matrix enzyme that catalyzes the oxidation of isovaleryl-CoA to 3-methylcrotonyl-CoA. …
NAID 130005072136

好塩性アーキアの膜脂質イソプレノイド生成機構-ロイシン-メバロン酸経路と関連酵素の存在

山内敬明,田上諒
日本地球化学会年会要旨集 60(0), 139, 2013
好塩性古細菌の特徴的なイソプレノイド脂質コア部分の生合成機構の詳細について，ロイシンからメバロン酸への短絡経路と関連酵素の存在を指摘するため，立体特異的重水素化標識ロイシンを用いて検討した。(3S)- および(3R)-[3-d]ロイシンを調製し，その取り込み実験より，プロキラル水素が立体特異的に変換されていることが示され，これは相当するイソバレリル-CoA 脱水素酵素が基質を立体特異的に認識してい …
NAID 130004593890

超好熱アーキアの新規なNAD(P)依存性アミノ酸脱水素酵素の機能と構造解析

米田一成
ビタミン 86(2), 74-83, 2012-02-25
… In contrast to abundant information available about glutamate, alanine, leucine and phenylalanine dehydrogenases, information about the structure and function of lysine, aspartate, threonine dehydrogenases has been rather limited so far. …
NAID 110009419212

「ロイシン脱水素酵素」

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leucine dehydrogenase
Identifiers
EC number	1.4.1.9
CAS number	9082-71-7
Databases
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway
PRIAM	profile
PDB structures	RCSB PDB PDBe PDBsum
Gene Ontology	AmiGO / EGO
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NCBI	proteins