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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/20 05:57:39」(JST)
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| glutathione synthetase |
| Identifiers |
| Symbol |
GSS |
| Entrez |
2937 |
| HUGO |
4624 |
| OMIM |
601002 |
| RefSeq |
NM_000178 |
| UniProt |
P48637 |
| Other data |
| EC number |
6.3.2.3 |
| Locus |
Chr. 20 q11.2 |
| Eukaryotic glutathione synthase |
|
human glutathione synthetase
|
| Identifiers |
| Symbol |
GSH_synthase |
| Pfam |
PF03199 |
| Pfam clan |
CL0483 |
| InterPro |
IPR004887 |
| SCOP |
2hgs |
| SUPERFAMILY |
2hgs |
| Available protein structures: |
| Pfam |
structures |
| PDB |
RCSB PDB; PDBe; PDBj |
| PDBsum |
structure summary |
|
| Eukaryotic glutathione synthase, ATP binding domain |
|
human glutathione synthetase
|
| Identifiers |
| Symbol |
GSH_synth_ATP |
| Pfam |
PF03917 |
| InterPro |
IPR005615 |
| SCOP |
1m0t |
| SUPERFAMILY |
1m0t |
| Available protein structures: |
| Pfam |
structures |
| PDB |
RCSB PDB; PDBe; PDBj |
| PDBsum |
structure summary |
|
| Prokaryotic glutathione synthetase, N-terminal domain |
|
structure of escherichia coli glutathione synthetase at ph 7.5
|
| Identifiers |
| Symbol |
GSH-S_N |
| Pfam |
PF02951 |
| InterPro |
IPR004215 |
| SCOP |
1glv |
| SUPERFAMILY |
1glv |
| Available protein structures: |
| Pfam |
structures |
| PDB |
RCSB PDB; PDBe; PDBj |
| PDBsum |
structure summary |
|
| Prokaryotic glutathione synthetase, ATP-grasp domain |
|
structure of escherichia coli glutathione synthetase at ph 7.5
|
| Identifiers |
| Symbol |
GSH-S_ATP |
| Pfam |
PF02955 |
| Pfam clan |
CL0179 |
| InterPro |
IPR004218 |
| SCOP |
1glv |
| SUPERFAMILY |
1glv |
| Available protein structures: |
| Pfam |
structures |
| PDB |
RCSB PDB; PDBe; PDBj |
| PDBsum |
structure summary |
|
Glutathione synthetase (GSS) (EC 6.3.2.3) is the second enzyme in the glutathione biosynthesis pathway. It catalyses the condensation of gamma-glutamylcysteine and glycine, to form glutathione.[1]
In eukaryotes, this is a homodimeric enzyme. In humans, defects in GSS are inherited in an autosomal recessive way and are the cause of severe metabolic acidosis, 5-oxoprolinuria, and increased rate of haemolysis and defective function of the central nervous system. The substrate-binding domain has a 3-layer alpha/beta/alpha structure.[2]
See also
- Glutathione synthetase deficiency
References
- ^ Njålsson R, Norgren S (2005). "Physiological and pathological aspects of GSH metabolism.". Acta Paediatr 94 (2): 132–7. doi:10.1080/08035250410025285. PMID 15981742.
- ^ Polekhina G, Board PG, Gali RR, Rossjohn J, Parker MW (June 1999). "Molecular basis of glutathione synthetase deficiency and a rare gene permutation event". EMBO J. 18 (12): 3204–13. doi:10.1093/emboj/18.12.3204. PMC 1171401. PMID 10369661.
External links
- Glutathione Synthetase at the US National Library of Medicine Medical Subject Headings (MeSH)
|
Enzymes: CO CS and CN ligases (EC 6.1-6.3)
|
|
| 6.1: Carbon-Oxygen |
- Aminoacyl tRNA synthetase
- Alanine
- Arginine
- Asparagine
- Aspartate
- Cysteine
- D-alanine—poly(phosphoribitol) ligase
- Glutamate
- Glutamine
- Glycine
- Histidine
- Isoleucine
- Leucine
- Lysine
- Methionine
- Phenylalanine
- Proline
- Serine
- Threonine
- Tryptophan
- Tyrosine
- Valine
|
|
| 6.2: Carbon-Sulfur |
- Succinyl coenzyme A synthetase
- Acetyl—CoA synthetase
- Long-chain-fatty-acid—CoA ligase
|
|
| 6.3: Carbon-Nitrogen |
- Glutamine synthetase
- Ubiquitin ligase
- Cullin
- Von Hippel–Lindau tumor suppressor
- UBE3A
- Mdm2
- Anaphase-promoting complex
- UBR1
- Glutathione synthetase
- CTP synthetase
- Adenylosuccinate synthase
- Argininosuccinate synthase
- Holocarboxylase synthetase
- GMP synthase
- Asparagine synthetase
- Carbamoyl phosphate synthetase
- Glutamate–cysteine ligase
|
|
- Biochemistry overview
- Enzymes overview
- By EC number: 1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
- 2.1
- 3.1
- 4.1
- 5.1
- 6.1-3
|
|
|
|
|
Ligases: carbon-carbon ligases (EC 6.4)
|
|
| Biotin dependent carboxylation |
- Pyruvate carboxylase
- Acetyl-CoA carboxylase
- Propionyl-CoA carboxylase
- Methylcrotonyl-CoA carboxylase
|
|
| Other |
|
|
- Biochemistry overview
- Enzymes overview
- By EC number: 1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
- 2.1
- 3.1
- 4.1
- 5.1
- 6.1-3
|
|
|
|
|
Enzymes: Phosphoric ester and nitrogen-metal ligases (EC 6.5-6.6)
|
|
| 6.5: Phosphoric Ester |
|
|
| 6.6: Nitrogen-Metal |
- Magnesium chelatase
- Cobalt chelatase
|
|
- Biochemistry overview
- Enzymes overview
- By EC number: 1.1
- 2
- 3
- 4
- 5
- 6
- 7
- 8
- 10
- 11
- 13
- 14
- 15-18
- 2.1
- 3.1
- 4.1
- 5.1
- 6.1-3
|
|
|
|
|
Metabolism: Protein metabolism, synthesis and catabolism enzymes
|
|
|
Essential amino acids are in Capitals
|
|
| K→acetyl-CoA |
| LYSINE→ |
- Saccharopine dehydrogenase
- Glutaryl-CoA dehydrogenase
|
|
| LEUCINE→ |
- Branched chain aminotransferase
- Branched-chain alpha-keto acid dehydrogenase complex
- Isovaleryl coenzyme A dehydrogenase
- Methylcrotonyl-CoA carboxylase
- Methylglutaconyl-CoA hydratase
- 3-hydroxy-3-methylglutaryl-CoA lyase
|
|
| TRYPTOPHAN→ |
- Indoleamine 2,3-dioxygenase/Tryptophan 2,3-dioxygenase
- Arylformamidase
- Kynureninase
- 3-hydroxyanthranilate oxidase
- Aminocarboxymuconate-semialdehyde decarboxylase
- Aminomuconate-semialdehyde dehydrogenase
|
|
| PHENYLALANINE→tyrosine→ |
|
|
|
| G |
G→pyruvate
→citrate |
| glycine→serine→ |
- Serine hydroxymethyltransferase
- Serine dehydratase
- glycine→creatine: Guanidinoacetate N-methyltransferase
- Creatine kinase
|
|
| alanine→ |
|
|
| cysteine→ |
|
|
| threonine→ |
- L-threonine dehydrogenase
|
|
|
G→glutamate→
α-ketoglutarate |
| HISTIDINE→ |
- Histidine ammonia-lyase
- Urocanate hydratase
- Formiminotransferase cyclodeaminase
|
|
| proline→ |
- Proline oxidase
- Pyrroline-5-carboxylate reductase
- 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1
- PYCR1
|
|
| arginine→ |
- Ornithine aminotransferase
- Ornithine decarboxylase
- Agmatinase
|
|
| →alpha-ketoglutarate→TCA |
|
|
| Other |
- cysteine+glutamate→glutathione: Gamma-glutamylcysteine synthetase
- Glutathione synthetase
- Gamma-glutamyl transpeptidase
- glutamate→glutamine: Glutamine synthetase
- Glutaminase
|
|
|
G→propionyl-CoA→
succinyl-CoA |
| VALINE→ |
- Branched chain aminotransferase
- Branched-chain alpha-keto acid dehydrogenase complex
- Enoyl-CoA hydratase
- 3-hydroxyisobutyryl-CoA hydrolase
- 3-hydroxyisobutyrate dehydrogenase
- Methylmalonate semialdehyde dehydrogenase
|
|
| ISOLEUCINE→ |
- Branched chain aminotransferase
- Branched-chain alpha-keto acid dehydrogenase complex
- 3-hydroxy-2-methylbutyryl-CoA dehydrogenase
|
|
| METHIONINE→ |
- generation of homocysteine: Methionine adenosyltransferase
- Adenosylhomocysteinase
- regeneration of methionine: Methionine synthase/Homocysteine methyltransferase
- Betaine-homocysteine methyltransferase
- conversion to cysteine: Cystathionine beta synthase
- Cystathionine gamma-lyase
|
|
| THREONINE→ |
|
|
| →succinyl-CoA→TCA |
- Propionyl-CoA carboxylase
- Methylmalonyl CoA epimerase
- Methylmalonyl-CoA mutase
|
|
|
| G→fumarate |
| PHENYLALANINE→tyrosine→ |
- Phenylalanine hydroxylase
- Tyrosine aminotransferase
- 4-Hydroxyphenylpyruvate dioxygenase
- Homogentisate 1,2-dioxygenase
- Fumarylacetoacetate hydrolase
- tyrosine→melanin: Tyrosinase
|
|
|
| G→oxaloacetate |
| asparagine→aspartate→ |
- Asparaginase/Asparagine synthetase
- Aspartate transaminase
|
|
|
|
|
Index of inborn errors of metabolism
|
|
| Description |
- Metabolism
- Enzymes and pathways: citric acid cycle
- pentose phosphate
- glycoproteins
- glycosaminoglycans
- phospholipid
- cholesterol and steroid
- sphingolipids
- eicosanoids
- amino acid
- urea cycle
- nucleotide
|
|
| Disorders |
- Citric acid cycle and electron transport chain
- Glycoprotein
- Proteoglycan
- Fatty-acid
- Phospholipid
- Cholesterol and steroid
- Eicosanoid
- Amino acid
- Purine-pyrimidine
- Heme metabolism
- Symptoms and signs
|
|
| Treatment |
|
|
|
UpToDate Contents
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English Journal
- Glycine restores glutathione and protects against oxidative stress in vascular tissue from sucrose-fed rats.
- Ruiz-Ramírez A, Ortiz-Balderas E, Cardozo-Saldaña G, Diaz-Diaz E, El-Hafidi M.Source*Department of Cardiovascular Biomedicine, Instituto Nacional de Cardiología 'Ignacio Chávez', Juan Badiano 1, Col. Sección XVI, Mexico City, D.F. 14080, Mexico.
- Clinical science (London, England : 1979).Clin Sci (Lond).2014 Jan 1;126(1):19-29. doi: 10.1042/CS20130164.
- The attenuation of oxidative stress could be an important mechanism whereby the incidence of vascular complications in the MS (metabolic syndrome) may be diminished. The present study was undertaken to investigate the mechanism by which glycine, supplemented to the diet of SF (sucrose-fed) rats, mod
- PMID 23742196
- Hepatoprotective effects of eburicoic acid and dehydroeburicoic acid from Antrodia camphorata in a mouse model of acute hepatic injury.
- Huang GJ, Deng JS, Huang SS, Lee CY, Hou WC, Wang SY, Sung PJ, Kuo YH.SourceDepartment of Chinese Pharmaceutical Sciences and Chinese Medicine Resources, College of Pharmacy, China Medical University, Taichung 404, Taiwan.
- Food chemistry.Food Chem.2013 Dec 1;141(3):3020-7. doi: 10.1016/j.foodchem.2013.03.061. Epub 2013 Apr 6.
- The hepatoprotective effects of eburicoic acid (TR1) and dehydroeburicoic acid (TR2) from Antrodia camphorata (AC) against carbon tetrachloride (CCl4)-induced liver damage were investigated in mice. TR1 and TR2 was administered intraperitoneally (i.p.) for 7 days prior to the administration of CCl4.
- PMID 23871054
Japanese Journal
- Znと化粧品 : 紫外線による色素沈着及びシワ形成抑制効果を中心に
- 正木 仁
- 薬学雑誌 132(3), 261-269, 2012
- … Zn2+ is well-known as an inducer of MT (metallothionein) and γGCS (γ-glutamyl cysteinyl synthetase: a rate-limiting enzyme of glutathione synthesis) via the up-regulation of their mRNAs through a metal transcription factor. … The inductions of both MT and glutathione are expected to reduce oxidative stress due to the more effective scavenging of intracellular ROS. …
- NAID 130001872019
- 分裂酵母に誘導合成される重金属結合ペプチド:Cadystin(Phytochelatin) : 生合成の機構,光反応性半導体製造への応用,および重金属環境汚染検出への利用
- 村杉 章
- 生物工学会誌 : seibutsu-kogaku kaishi 89(5), 228-237, 2011-05-25
- 分裂酵母をカドミウムイオンに暴露すると多量のカドミウム-ペプチド複合体が誘導合成される.主要な複合体はカドミウム,cadystinと名づけられたペプチド,および酸不安定硫黄で構成されている.Cadystinおよび酸不安定硫黄は,この種の複合体中に見いだされたのは初めてである.Cadystinの主要成分の化学構造は,γ-Glu-Cys-γ-Glu-Cys-γ-Glu-Cys-Gly,およびγ-Glu …
- NAID 110008662242
Related Links
- Glutathione synthetase deficiency is a disorder that prevents the production of an important molecule called glutathione. Glutathione helps prevent damage to cells by neutralizing harmful molecules generated during ...
- Isoform 1 (identifier: P48637-1) [UniParc]FASTA Add to basket Added to basket This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in ...
Related Pictures
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★リンクテーブル★
[★]
- 英
- glutathione synthetase、glutathione synthase
- 関
- グルタチオンシンターゼ、グルタチオンシンセターゼ、グルタチオンシンテターゼ
[★]
- 英
- glutathione synthetase
- 関
- グルタチオン合成酵素、グルタチオンシンテターゼ
[★]
- 英
- glutathione synthetase
- 関
- グルタチオン合成酵素、グルタチオンシンセターゼ
[★]
- 関
- ligase、synthase