葡萄糖-6-磷酸脫氫酶

葡萄糖-6-磷酸脫氫酶
	葡萄糖-6-磷酸脫氫酶結構（PDB 1qki）
命名
系統命名	D-glucose-6-phosphate:NADP+ 1-oxidoreductase
縮寫	G6PDH; GPD
別名	NADP-glucose-6-phosphate dehydrogenase; Zwischenferment; D-glucose 6-phosphate dehydrogenase; glucose 6-phosphate dehydrogenase (NADP); NADP-dependent glucose 6-phosphate dehydrogenase; 6-phosphoglucose dehydrogenase; Entner-Doudoroff enzyme; glucose-6-phosphate 1-dehydrogenase
識別碼
EC編號	1.1.1.49
CAS號	9001-40-5
數據庫
IntEnz	IntEnz瀏覽
BRENDA（英語：BRENDA）	BRENDA入口
ExPASy（英語：ExPASy）	NiceZyme瀏覽
KEGG	KEGG入口
MetaCyc（英語：MetaCyc）	代謝路徑
PRIAM（英語：PRIAM_enzyme-specific_profiles）	概述
PDB	RCSB PDB PDBj PDBe PDBsum
基因本體	AmiGO / EGO
搜尋
PMC	相關文獻
PubMed	相關文獻

葡萄糖-6-磷酸脫氫酶
已知的結構
PDB	直系同源搜尋: PDBe RCSB
PDBID列表
	1QKI、2BH9、2BHL
識別號
別名	G6PD；, G6PD1, glucose-6-phosphate dehydrogenase, Glucose-6-phosphate dehydrogenase
外部ID	OMIM：305900 MGI：105977 HomoloGene：37906 GeneCards：G6PD
相關疾病
	貧血
基因位置（人類）
染色體	X染色體
終止	154,547,572 bp
基因位置（小鼠）
染色體	小鼠5號染色體
終止	61,968,506 bp
RNA表達模式
	查閱更多表達數據
基因本體
分子功能	• protein homodimerization activity; • glucose binding; • 血漿蛋白結合; • NADP binding; • 相同蛋白質結合; • oxidoreductase activity; • carbohydrate binding; • glucose-6-phosphate dehydrogenase activity; • oxidoreductase activity, acting on CH-OH group of donors;
細胞組分	• 細胞質; • 細胞質基質; • 膜; • cytoplasmic side of plasma membrane; • 外排體; • 細胞核; • 細胞內膜結合細胞器; • 微管組織中心;
生物學過程	• NADP metabolic process; • response to organic cyclic compound; • substantia nigra development; • glutathione metabolic process; • NADPH regeneration; • pentose-phosphate shunt, oxidative branch; • 脂質代謝; • regulation of neuron apoptotic process; • ribose phosphate biosynthetic process; • pentose biosynthetic process; • negative regulation of protein glutathionylation; • 磷酸戊糖途徑; • glucose metabolic process; • response to food; • cellular response to oxidative stress; • cholesterol biosynthetic process; • glucose 6-phosphate metabolic process; • response to ethanol; • erythrocyte maturation; • positive regulation of calcium ion transmembrane transport via high voltage-gated calcium channel; • negative regulation of reactive oxygen species metabolic process; • negative regulation of cell growth involved in cardiac muscle cell development; • response to iron(III) ion; • 碳水化合物代謝;
	Sources:Amigo / QuickGO
直系同源
	2539
	14380
	ENSG00000160211
	ENSMUSG00000089992
	P11413
	P97324
	NM_000402; NM_001042351; NM_001360016
	NM_019468
	NP_000393; NP_001035810; NP_001346945
	NP_062341
	維基數據
檢視/編輯人類	檢視/編輯小鼠

葡萄糖-6-磷酸脫氫酶（英語：glucose-6-phosphate dehydrogenase，EC 1.1.1.49 （頁面存檔備份，存於互聯網檔案館））是一種以NAD⁺或NADP⁺為受體、作用於供體 CH-OH 基團上的氧化還原酶。這種酶能催化以下酶促反應：

D-葡萄糖-6-磷酸 + NADP⁺

\rightleftharpoons

D-葡萄糖酸-1,5-內酯-6-磷酸 + NADPH + H⁺

葡萄糖-6-磷酸脫氫酶主要參與磷酸戊糖途徑，也能緩慢地作用在β-D-葡萄糖等其他糖類上。這種酶可由其基質葡萄糖-6-磷酸活化。

葡萄糖-6-磷酸脫氫酶存在於眾多生物細胞內，高等植物擁有數種葡萄糖-6-磷酸脫氫酶的蛋白異構體（protein isoform），它們分佈於細胞質基質、和過氧化物酶體等細胞結構中^[6]。如果人類表達葡萄糖-6-磷酸脫氫酶的基因有缺陷，將會導致葡萄糖-6-磷酸脫氫酶缺乏症，進而引發非免疫性溶血性貧血（英語：Hemolytic anemia）。

物種分佈

從細菌基因組到人類基因組中都有G6PD。一項對百餘個物種的G6PD多重序列比對研究顯示，不同物種間的G6PD基因相似度在30%到94%之間^[7]，人與細菌間的相似度有30%^[8]。

人類的G6PD基因存在兩種蛋白異構體^[9]，此基因上至少有168種與疾病有關的突變^[10]，通常為改變氨基酸的錯義突變^[11]。

參考資料

^ 與葡萄糖-6-磷酸脱氢酶相關的疾病；在維基數據上查看/編輯參考.
^ ^2.0 ^2.1 ^2.2 GRCh38: Ensembl release 89: ENSG00000160211 - Ensembl, May 2017
^ ^3.0 ^3.1 ^3.2 GRCm38: Ensembl release 89: ENSMUSG00000089992 - Ensembl, May 2017
^ Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Corpas FJ; et al. A dehydrogenase-mediated recycling system of NADPH in plant peroxisomes. Biochem. J. 1998, 330 (7): 777–784.
^ Kotaka M, Gover S, Vandeputte-Rutten L, Au SW, Lam VM, Adams MJ. Structural studies of glucose-6-phosphate and NADP+ binding to human glucose-6-phosphate dehydrogenase (PDF). Acta Crystallographica D. May 2005, 61 (Pt 5): 495–504. PMID 15858258. doi:10.1107/S0907444905002350  .
^ Au SW, Gover S, Lam VM, Adams MJ. Human glucose-6-phosphate dehydrogenase: the crystal structure reveals a structural NADP(+) molecule and provides insights into enzyme deficiency. Structure. March 2000, 8 (3): 293–303. PMID 10745013. doi:10.1016/S0969-2126(00)00104-0  .
^ G6PD glucose-6-phosphate dehydrogenase [ Homo sapiens (human) ]. NCBI. [13 December 2015].
^ Šimčíková D, Heneberg P. Refinement of evolutionary medicine predictions based on clinical evidence for the manifestations of Mendelian diseases. Scientific Reports. December 2019, 9 (1): 18577. Bibcode:2019NatSR...918577S. PMC 6901466  . PMID 31819097. doi:10.1038/s41598-019-54976-4.
^ Kiani F, Schwarzl S, Fischer S, Efferth T. Three-dimensional modeling of glucose-6-phosphate dehydrogenase-deficient variants from German ancestry. PLOS ONE. July 2007, 2 (7): e625. Bibcode:2007PLoSO...2..625K. PMC 1913203  . PMID 17637841. doi:10.1371/journal.pone.0000625  .

延伸閱讀

Vulliamy T, Beutler E, Luzzatto L. Variants of glucose-6-phosphate dehydrogenase are due to missense mutations spread throughout the coding region of the gene.. Hum. Mutat. 1993, 2 (3): 159–67. PMID 8364584. doi:10.1002/humu.1380020302.

Mason PJ. New insights into G6PD deficiency.. Br. J. Haematol. 1996, 94 (4): 585–91. PMID 8826878.

Wajcman H, Galactéros F. [Glucose 6-phosphate dehydrogenase deficiency: a protection against malaria and a risk for hemolytic accidents]. C. R. Biol. 2004, 327 (8): 711–20. PMID 15506519.

外部連結

[1] 與葡萄糖-6-磷酸脱氢酶相關的疾病；在維基數據上查看/編輯參考.

[refGRCh38Ensembl-2] 2.0 ^2.1 ^2.2 GRCh38: Ensembl release 89: ENSG00000160211 - Ensembl, May 2017

[refGRCm38Ensembl-3] 3.0 ^3.1 ^3.2 GRCm38: Ensembl release 89: ENSMUSG00000089992 - Ensembl, May 2017

[4] Human PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.

[5] Mouse PubMed Reference:. National Center for Biotechnology Information, U.S. National Library of Medicine.

[6] Corpas FJ; et al. A dehydrogenase-mediated recycling system of NADPH in plant peroxisomes. Biochem. J. 1998, 330 (7): 777–784.

[:1-7] Kotaka M, Gover S, Vandeputte-Rutten L, Au SW, Lam VM, Adams MJ. Structural studies of glucose-6-phosphate and NADP+ binding to human glucose-6-phosphate dehydrogenase (PDF). Acta Crystallographica D. May 2005, 61 (Pt 5): 495–504. PMID 15858258. doi:10.1107/S0907444905002350  .

[:2-8] Au SW, Gover S, Lam VM, Adams MJ. Human glucose-6-phosphate dehydrogenase: the crystal structure reveals a structural NADP(+) molecule and provides insights into enzyme deficiency. Structure. March 2000, 8 (3): 293–303. PMID 10745013. doi:10.1016/S0969-2126(00)00104-0  .

[9] G6PD glucose-6-phosphate dehydrogenase [ Homo sapiens (human) ]. NCBI. [13 December 2015].

[Šimčíková_2019_-_supplementary_table_S7-10] Šimčíková D, Heneberg P. Refinement of evolutionary medicine predictions based on clinical evidence for the manifestations of Mendelian diseases. Scientific Reports. December 2019, 9 (1): 18577. Bibcode:2019NatSR...918577S. PMC 6901466  . PMID 31819097. doi:10.1038/s41598-019-54976-4.

[:0-11] Kiani F, Schwarzl S, Fischer S, Efferth T. Three-dimensional modeling of glucose-6-phosphate dehydrogenase-deficient variants from German ancestry. PLOS ONE. July 2007, 2 (7): e625. Bibcode:2007PLoSO...2..625K. PMC 1913203  . PMID 17637841. doi:10.1371/journal.pone.0000625  .

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