glucose-6-phosphate dehydrogenase

グルコース6-リン酸脱水素酵素 G6PD

WordNet

1. a salt of phosphoric acid (同)orthophosphate, inorganic_phosphate
2. carbonated drink with fruit syrup and a little phosphoric acid
3. a monosaccharide sugar that has several forms; an important source of physiological energy

PrepTutorEJDIC

1. 〈U〉リン酸塩 / 《複数形で》リン酸肥料
2. ブドウ糖

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/10 16:11:04」(JST)

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Glucose-6-phosphate dehydrogenase
Identifiers
EC number	1.1.1.49
CAS number	9001-40-5
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
Search PMC articles PubMed articles NCBI proteins

Glucose-6-phosphate dehydrogenase, NAD binding domain
glucose 6-phosphate dehydrogenase from leuconostoc mesenteroides
Identifiers
Symbol	G6PD_N
Pfam	PF00479
Pfam clan	CL0063
InterPro	IPR022674
PROSITE	PDOC00067
SCOP	1dpg
SUPERFAMILY	1dpg
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Glucose-6-phosphate dehydrogenase, C-terminal domain
Identifiers
Symbol	G6PD_C
Pfam	PF02781
PROSITE	PDOC00067
SCOP	1dpg
SUPERFAMILY	1dpg
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Glucose-6-phosphate dehydrogenase
PDB rendering based on 1qki.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1QKI, 2BH9, 2BHL
Identifiers
Symbols	G6PD ; G6PD1
External IDs	OMIM: 305900 MGI: 105979 HomoloGene: 37906 ChEMBL: 5347 GeneCards: G6PD Gene
EC number	1.1.1.49
Gene ontology Molecular function • glucose-6-phosphate dehydrogenase activity • protein binding • glucose binding • protein homodimerization activity • NADP binding Cellular component • nucleus • cytoplasm • cytosol • cytoplasmic side of plasma membrane • membrane • extracellular vesicular exosome Biological process • cytokine production • carbohydrate metabolic process • pentose-phosphate shunt • lipid metabolic process • cholesterol biosynthetic process • NADP metabolic process • NADPH regeneration • glutathione metabolic process • pentose-phosphate shunt, oxidative branch • negative regulation of protein glutathionylation • response to organic cyclic compound • pentose biosynthetic process • substantia nigra development • response to food • cellular response to oxidative stress • erythrocyte maturation • regulation of neuron apoptotic process • small molecule metabolic process • response to ethanol • ribose phosphate biosynthetic process • glucose 6-phosphate metabolic process • oxidation-reduction process Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	2539	14381
Ensembl	ENSG00000160211	ENSMUSG00000031400
UniProt	P11413	Q00612
RefSeq (mRNA)	NM_000402	NM_008062
RefSeq (protein)	NP_000393	NP_032088
Location (UCSC)	Chr HG1497_PATCH: 153.7 – 153.72 Mb	Chr X: 74.41 – 74.43 Mb
PubMed search	[1]	[2]

Glucose-6-phosphate dehydrogenase (G6PD or G6PDH) (EC 1.1.1.49) is a cytosolic enzyme that catalyzes the chemical reaction

D-glucose 6-phosphate + NADP⁺ 6-phospho-D-glucono-1,5-lactone + NADPH + H⁺

This enzyme is in the pentose phosphate pathway (see image), a metabolic pathway that supplies reducing energy to cells (such as erythrocytes) by maintaining the level of the co-enzyme nicotinamide adenine dinucleotide phosphate (NADPH). The NADPH in turn maintains the level of glutathione in these cells that helps protect the red blood cells against oxidative damage. Of greater quantitative importance is the production of NADPH for tissues actively engaged in biosynthesis of fatty acids and/or isoprenoids, such as the liver, mammary glands, adipose tissue, and the adrenal glands. G6PD reduces nicotinamide adenine dinucleotide phosphate (NADP) to NADPH while oxidizing glucose-6-phosphate.

It is notable in humans when there is a genetic deficiency of G6PD which predisposes to non-immune hemolytic anemia .

Species distribution

G6PD is widely distributed in many species from bacteria to humans. In higher plants, several isoforms of G6PDH have been reported, which are localized in the cytosol, the plastidic stroma, and peroxisomes. Among humans, G6PD is common in certain insular groups, such as Parsis.

Regulation

Glucose-6-phosphate dehydrogenase is stimulated by its substrate Glucose 6 Phosphate. The usual ratio of NADPH/NADP⁺ in the cytosol of tissues engaged in biosyntheses is about 100/1. Increased utilization of NADPH for fatty acid biosynthesis will dramatically increase the level of NADP⁺, thus stimulating G6PD to produce more NADPH.

G6PD converts glucose-6-phosphate into 6-phosphoglucono-δ-lactone and is the rate-limiting enzyme of the pentose phosphate pathway.

G6PD is one of a number of glycolytic enzymes activated by the transcription factor Hypoxia-inducible factor 1 (HIF1).

Clinical significance

G6PD is remarkable for its genetic diversity. Many variants of G6PD, mostly produced from missense mutations, have been described with wide ranging levels of enzyme activity and associated clinical symptoms. Two transcript variants encoding different isoforms have been found for this gene.

Glucose-6-phosphate dehydrogenase deficiency is very common worldwide, and causes acute hemolytic anemia in the presence of simple infection, ingestion of fava beans, or reaction with certain medicines, antibiotics, antipyretics, and antimalarials.

Cell growth and proliferation are affected by G6PD. G6PD inhibitors are under investigation to treat cancers and other conditions. DHEA is a G6PD inhibitor.

List of medicines which cause lysis in G6PD

Note that this list doesn't contain all the medicines which cause hemolysis:

• Antipyretics
• Acetamilide
• Acetophenetidin
• Antipyrine
• Aspirin
• Antimalarials
• Chloroquine
• Primaquine
• Pamaquine
• Quinocide
• Plasmoquine
• Sulfonamides
• Sulfamilamide
• SulfaCetamide
• Sulfapyridine
• Salicyayo sulfapyridine
• N2 acetyl sulfanilamide
• Nitrofurantoin
• Furaltadone
• Furazolidone
• Nitrofurazone
• Sulfuae
• Sulfoxone
• Thiazolsulfone
• Dapsone
• Dimercaprol
• Orianse
• Neo salvarsan
• Qulndene

See also

• Glucose-6-phosphate dehydrogenase deficiency
• Genetic resistance to malaria

References

Further reading

• Vulliamy T, Beutler E, Luzzatto L (1993). "Variants of glucose-6-phosphate dehydrogenase are due to missense mutations spread throughout the coding region of the gene". Hum. Mutat. 2 (3): 159–67. doi:10.1002/humu.1380020302. PMID 8364584. 
• Mason PJ (1996). "New insights into G6PD deficiency". Br. J. Haematol. 94 (4): 585–91. PMID 8826878. 
• Wajcman H, Galactéros F (2004). "[Glucose 6-phosphate dehydrogenase deficiency: a protection against malaria and a risk for hemolytic accidents]". C. R. Biol. 327 (8): 711–20. doi:10.1016/j.crvi.2004.07.010. PMID 15506519. 

External links

• - G6PD Deficiency Website
• ATSDR - G6PD Deficiency

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 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. 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
• 3. 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
• 4. ヘキソースリン酸側路およびグルタチオン代謝疾患（グルコース-6-リン酸脱水素酵素欠損症を除く） disorders of the hexose monophosphate shunt and glutathione metabolism other than glucose 6 phosphate dehydrogenase deficiency
• 5. Drug-induced thrombotic microangiopathy

English Journal

• Expressional regulation of key hepatic enzymes of intermediary metabolism in European seabass (Dicentrarchus labrax) during food deprivation and refeeding.

• Viegas I1, Caballero-Solares A2, Rito J3, Giralt M4, Pardal MA5, Metón I4, Jones JG6, Baanante IV4.
• Comparative biochemistry and physiology. Part A, Molecular & integrative physiology.Comp Biochem Physiol A Mol Integr Physiol.2014 Aug;174:38-44. doi: 10.1016/j.cbpa.2014.04.004. Epub 2014 Apr 18.
• We hypothesized that the analysis of mRNA level and activity of key enzymes in amino acid and carbohydrate metabolism in a feeding/fasting/refeeding setting could improve our understanding of how a carnivorous fish, like the European seabass (Dicentrarchus labrax), responds to changes in dietary int
• PMID 24746983

• Prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency in the Ouest and Sud-Est departments of Haiti.

• von Fricken ME1, Weppelmann TA2, Eaton WT3, Alam MT4, Carter TE5, Schick L6, Masse R7, Romain JR8, Okech BA9.
• Acta tropica.Acta Trop.2014 Jul;135:62-6. doi: 10.1016/j.actatropica.2014.03.011. Epub 2014 Mar 26.
• Malaria remains a significant public health issue in Haiti, with chloroquine (CQ) used almost exclusively for the treatment of uncomplicated infections. Recently, single dose primaquine (PQ) was added to the Haitian national malaria treatment policy, despite a lack of information on the prevalence o
• PMID 24681219

• Oxidative stress and suicidal erythrocyte death.

• Lang F1, Abed M, Lang E, Föller M.
• Antioxidants & redox signaling.Antioxid Redox Signal.2014 Jul 1;21(1):138-53. doi: 10.1089/ars.2013.5747. Epub 2014 Apr 1.
• Abstract Significance: Eryptosis, the suicidal erythrocyte death, is characterized by cell shrinkage, membrane blebbing, and phosphatidylserine translocation to the outer membrane leaflet. Phosphatidylserine at the erythrocyte surface binds endothelial CXCL16/SR-PSOX (CXC-Motiv-Chemokin-16/Scavenger
• PMID 24359125

Japanese Journal

• 智歯周囲炎から溶血発作を生じたG6PD異常症の1例

• 五島 秀樹,清水 武,川原 理絵
• 日本口腔外科学会雑誌 58(6), 376-380, 2012-06
• NAID 40019370377

• 自治医科大学医動物学教室で4年間(2007-2010)に経験した寄生虫・衛生動物関連症例の検討

• 松岡 裕之,山本 大介,早川 枝李,諏合 輝子,佐野 元市郎,平井 誠,吉田 栄人
• 自治医科大学紀要 34, 117-128, 2012-03-01
• … 新生児の溶血性貧血またはマラリアの治療に関連してglucose-6-phosphate dehydrogenase (G6PD)活性検査の依頼が34件寄せられた。 … 新生児に強い黄疸が生じ母親が外国人である場合,G6PD欠損症を疑って本酵素の活性を検査するべきである。 …
• NAID 110008922665

• ブタの卵丘・卵母細胞複合体におけるグルコース-6-リン酸脱水素酵素活性と卵母細胞の核成熟との関係

• 鈴木 亮,新村 末雄
• 新潟大学農学部研究報告 64(1), 1-6, 2011-09
• NAID 40019056711

Related Links

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• Glucose-6-phosphate dehydrogenase deficiency ...

Causes G6PD deficiency occurs when a person is missing or doesn't have enough of an enzyme called glucose-6-phosphate dehydrogenase, which helps red blood cells work properly. Too little G6PD leads to the ...

Related Pictures

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★リンクテーブル★

リンク元	「解糖系」「グルコース6-リン酸」「ペントースリン酸経路」「代謝系」「グルコース6-リン酸脱水素酵素」
拡張検索	「glucose-6-phosphate dehydrogenase deficiency」「glucose-6-phosphate dehydrogenase欠乏症」
関連記事	「phosphate」「dehydrogenase」「dehydrogenases」

「解糖系」

　　[★]

英

glycolytic pathway

同

エムデン-マイヤーホフ経路 Embden-Meyerhof pathway

関

解糖、TCA回路

• CH(OH)-CH(OH)-C(OH)H-CH(OH)-C(CH2OH)H-O- グルコース (OHが↓↓↑↓↑)
• CH(OH)-CH(OH)-C(OH)H-CH(OH)-C(CH2O-PO3)H-O- グルコース-6-リン酸 (OHが↓↓↑↓↑)
• C(CH2OH)OH-C(OH)H-CH(OH)-C(CH2O-PO3)H-O- フルクトース-6-リン酸
• C(CH2O-PO3)OH-C(OH)H-CH(OH)-C(CH2O-PO3)H-O- フルクトース-1,6-リン酸

glucose
↓-hexokinase/glucokinase(liver)
glucose 6-phosphate
↓-phosphohexose isomerase
fructose 6-phosphate
↓-phosphofructokinase
fructose 1,6-bisphosphate
↓-aldolase
glyceraldehyde 3-phosphate
↓-glyceraldehyde-3-phosphate dehydrogenase
1,3-bisphosphoglycerate
↓-phosphoglycerate kinase →ATP
3-phosphoglycerate
↓-phosphoglyceate mutase
2-phosphoglycerate
↓-enolase
phosphoenolpyruvate
↓-pyruvate kinase → ATP
pyruvate
　－(pyruvate dehydrogenase)→acetyl-CoA
　－(pyruvate carboxylase)→oxaloacetate－(NADH+H+)→malate

---

解糖系の酵素 (first aid step1 2006 p.87, FASTEP1_87_酵素一覧.xls)

1	galactokinase	キナーゼ
2	galactose-1-phosphate uridyltransferase	転移酵素
3	hexokinase/glucokinase	キナーゼ
4	glucose-6-phosphatase	ホスファターゼ
5	glucose-6-phosphate dehydrogenase	脱水素酵素
6	transketolase
7	phosphofructokinase	キナーゼ
8	fructose-1,6-bisphosphatase	ホスファターゼ
9	fructokinase	キナーゼ	フルクトキナーゼ
10	aldolase B		アルドラーゼ
11	pyruvate kinase	キナーゼ	ピルビン酸キナーゼ
12	pyruvate dehydrogenase	脱水素酵素	ピルビン酸デヒドロゲナーゼ
13	HMG-CoA reductase	還元酵素	HMG-CoA還元酵素
14	pyruvate carboxylase	カルボキシラーゼ	ピルビン酸カルボキシラーゼ
15	PEP carboxykinase	キナーゼ	PEPカルボキシキナーゼ
16	citrate synthase	合成酵素	クエン酸合成酵素
17	α-ketoglutarate dehydrogenase	脱水素酵素	α-ケトグルタミン酸脱水素酵素
18	ornithine transcarbamylase	転移酵素	オルニチンカルバモイルトランスフェラーゼ

「グルコース6-リン酸」

　　[★]

英

glucose 6-phosphate, G6P, G-6-P

同

グルコース-6-リン酸 glucose-6-phosphate

関

グルコース1-リン酸、グルコース、解糖系、糖新生

グルコース6-リン酸の運命(G6P) (FB.451)

1. グルコース
2. グリコーゲン
3. リボース5-リン酸(R5P)
4. アセチルCoA

解糖

• glucose－→glucose-6-phosphate

酵素

• 筋肉：glucokinase
• 肝臓：hexokinase

• glucose-6-phosphate－(phosphohexose isomerase)→fructose-6-phosphate

グリコーゲン分解

• glycogen－([[]])→glucose 1-phosphate－([[]])→glucose 6-phosphate

糖新生

• fructose-1,6-bisphosphate－(fructose-1,6-bisphosphatase)→fructose-6-phosphate←([[]])→glucose-6-phosphate－(glucose-6-phosphatase)→glucose

臨床関連

• グルコース-6-リン酸脱水素酵素欠損症

• glucose-6-phosphate dehydrogenaseの欠損による

• フォン・ギールケ病

• glucose-6-phosphataseの欠損による

「ペントースリン酸経路」

　　[★]

英

pentose phosphate pathway, PPP, pentose phosphate cycle

同

五炭糖リン酸回路、ヘキソース一リン酸回路 hexose monophosphate pathway、hexose monophosphate shunt HMP shunt、ワールブルグ・ディケンズ経路 ワーブルグ・ディッケンズ経路 Warburg-Dickens pathway、ホスホグルコン酸経路？？ phosphogluconate oxidative pathway？？

関

解糖系

• NADPHとリボース5-リン酸を産生する経路

臨床関連

• グルコース-6-リン酸脱水素酵素欠損症 G6PD欠損症

• グルコース6-リン酸脱水素酵素 glucose-6-phosphate dehydrogenase

「代謝系」

　　[★]

英

metabolic pathway

関

代謝経路

• 解糖系
• TCA回路
• 電子伝達系
• 尿酸回路
• 糖新生経路

---

• 1 galactokinase
• 2 galactose-1-phosphate uridyltransferase
• 3 hexokinase/glucokinase
• 4 glucose-6-phosphatase
• 5 glucose-6-phosphate dehydrogenase
• 6 transketolase
• 7 phosphofructokinase
• 8 fructose-1,6-bisphosphatase
• 9 fructokinase
• 10 aldolase B
• 11 pyruvate kinase
• 12 pyruvate dehydrogenase
• 13 HMG-CoA reductase
• 14 pyruvate carboxylase
• 15 PEP carboxykinase
• 16 citrate synthase
• 17 α-ketoglutarate dehydrogenase
• 18 ornithine transcarbamylase

「グルコース6-リン酸脱水素酵素」

　　[★]

英

glucose-6-phosphate dehydrogenase,G6PD

同

グルコース6-リン酸デヒドロゲナーゼ

関

HMP shunt、ペントースリン酸経路

• rate limit enzyme
• HMP shuntのribulose-5-phosphateからfructose-6-phosphateに代わる反応に関わる (FIRST AID p.87)

この経路はNADPHの産生に重要

glucose 6-phosphate + NADP+ → 6-phosphogluconolactone + NADPH + H+

臨床関連

• グルコース-6-リン酸脱水素酵素欠損症

「glucose-6-phosphate dehydrogenase deficiency」

　　[★] グルコース-6-リン酸脱水素酵素欠損症

関

G6PD deficiency

同

G6PD

「glucose-6-phosphate dehydrogenase欠乏症」

　　[★] グルコース-6-リン酸脱水素酵素欠損症

「phosphate」

　　[★]

• n.

• (化合物)リン酸塩、リン酸エステル、ホスフェート、フォスフェート、リン酸

関

inorganic phosphate、orthophosphate、orthophosphoric acid、phospho、phosphoester、phosphoric、phosphoric acid、phosphoric acid ester、phosphorus

　 　 　

「dehydrogenase」

　　[★] 脱水素酵素 デヒドロゲナーゼ

「dehydrogenases」

　　[★] 脱水素酵素 デヒドロゲナーゼ