hERG

Potassium voltage-gated channel, subfamily H (eag-related), member 2
Potassium voltage-gated channel, subfamily H (eag-related), member 2
	PDB rendering of the N-terminal domain based on 1byw. This domain is just a very small piece of the total channel.
有效結構
PDB	直系同源檢索：PDBe, RCSB
PDB查詢代碼列表
	1BYW, 1UJL, 2L0W, 2L1M, 2L4R, 2LE7, 4HP9, 4HQA
標識
代號	KCNH2; ERG1; HERG; HERG1; Kv11.1; LQT2; SQT1
擴展標識	遺傳學：152427 鼠基因：1341722 同源基因：201 IUPHAR: Kv11.1 ChEMBL: 240 GeneCards: KCNH2 Gene
基因本體論描述
分子功能	· phosphorelay sensor kinase activity; · inward rectifier potassium channel activity; · voltage-gated potassium channel activity; · delayed rectifier potassium channel activity; · protein binding; · ubiquitin protein ligase binding; · identical protein binding; · protein homodimerization activity; · voltage-gated potassium channel activity involved in ventricular cardiac muscle cell action potential repolarization;
細胞成分	· nuclear envelope; · cytoplasm; · plasma membrane; · voltage-gated potassium channel complex; · cell surface;
生物過程	· phosphorelay signal transduction system; · regulation of heart rate by hormone; · synaptic transmission; · signal transduction by phosphorylation; · cellular response to drug; · regulation of membrane potential; · potassium ion homeostasis; · cardiac muscle contraction; · regulation of membrane repolarization; · regulation of ventricular cardiac muscle cell membrane repolarization; · potassium ion export; · potassium ion transmembrane transport; · ventricular cardiac muscle cell action potential; · membrane depolarization during action potential; · membrane repolarization during action potential; · membrane repolarization during cardiac muscle cell action potential; · regulation of heart rate by cardiac conduction; · regulation of potassium ion transmembrane transport; · negative regulation of potassium ion transmembrane transport; · positive regulation of potassium ion transmembrane transport; · negative regulation of potassium ion export;
	Sources: Amigo / QuickGO
RNA表達模式
	更多表達數據
直系同源體
物種	人類
Entrez	3757
Ensembl	ENSG00000055118
UniProt	Q12809
mRNA序列	NM_000238
蛋白序列	NP_000229
基因位置	Chr 7:; 150.64 – 150.68 Mb
PubMed查詢	[1]
	閱; 論; 編;

hERG (the human Ether-à-go-go-Related Gene)，人延遲整流鉀離子通道基因，是指基因KCNH2。該基因編碼的蛋白質被稱為 K_v11.1，是鉀離子通道(英語：potassium ion channel)的α亞基（英語：alpha subunit）。該基因編碼的鉀離子通道（有時簡單地使用'hERG'表示）最為著名的是其對心臟的電位活性，可協調心跳（即，hERG通道介導在心臟動作電位中延遲整流鉀電流--I_Kr的復極化）。當這個通道介導電流通過細胞膜的能力受到抑制或妥協讓步時，不論是由於藥物的作用還是某些家族的基因突變引發，^[1]，都會導致潛在的致命疾病——QT間期延長綜合症。若干臨床上成功的上市藥物有抑制hERG的趨勢，導致其在使用過程中也伴隨著猝死的藥物不良反應風險。因此，在藥物研發過程中要儘量避免藥物對抗靶標(英語：antitarget)的hERG抑制。^[2] hERG還跟神經系統的某些細胞功能的調節^[3] 以及白血病細胞癌症特性的建立和維持^[4]有關。

命名

1994年4月，威斯康星大學麥迪遜分校的傑夫（英語：Jeff Warmke)和巴里（英語：Barry Ganetzky）在論文中首次命名和描述了hERG基因。^[5] hERG基因是首次發現於果蠅的Ether-à-go-go基因的人類同源類似物。 Ether-à-go-go基因由現在任職於希望城國家醫學中心（英語：City of Hope National Medical Center）的威廉·d·卡普蘭在1960年代命名。當使用乙醚麻醉擁有 Ether-à-go-go基因突變的蒼蠅時，它們的腿就開始顫抖，就像當時流行於加利福尼亞西好萊塢的威士忌搖擺舞（英語：Whisky A Go-Go）夜總會的舞蹈。

Interactions

HERG和 YWHAE之間存在相互作用。^[6]

生物功能

參考文獻

^ Hedley PL; Jorgensen P; Schlamowitz S; Wangari, Romilda; et al. The genetic basis of long QT and short QT syndromes: a mutation update. Human Mutation. 2009, 30 (11): 1486–511. PMID 19862833. doi:10.1002/humu.21106.
^ Sanguinetti MC, Tristani-Firouzi M. hERG potassium channels and cardiac arrhythmia. Nature. March 2006, 440 (7083): 463–9. PMID 16554806. doi:10.1038/nature04710.
^ Chiesa N, Rosati B, Arcangeli A, Olivotto M, Wanke E. A novel role for HERG K+ channels: spike-frequency adaptation. J. Physiol. (Lond.). June 1997,. 501 ( Pt 2) (2): 313–8. PMC 1159479  . PMID 9192303. doi:10.1111/j.1469-7793.1997.313bn.x. Overholt JL, Ficker E, Yang T, Shams H, Bright GR, Prabhakar NR. Chemosensing at the carotid body. Involvement of a HERG-like potassium current in glomus cells. Adv. Exp. Med. Biol. 2000, 475: 241–8. PMID 10849664. doi:10.1007/0-306-46825-5_22.
^ Arcangeli A. Expression and role of hERG channels in cancer cells. Novartis Found. Symp. 2005, 266: 225–32; discussion 232–4. PMID 16050271. doi:10.1002/047002142X.ch17.
^ Warmke JW, Ganetzky B. A family of potassium channel genes related to eag in Drosophila and mammals. Proc. Natl. Acad. Sci. U.S.A. April 1994, 91 (8): 3438–42 [2015-02-09]. PMC 43592  . PMID 8159766. doi:10.1073/pnas.91.8.3438. （原始內容存檔於2019-10-17）.
^ Kagan, Anna; Melman Yonathan F; Krumerman Andrew; McDonald Thomas V. 14-3-3 amplifies and prolongs adrenergic stimulation of HERG K+ channel activity. EMBO J. (England). Apr 2002, 21 (8): 1889–98. ISSN 0261-4189. PMC 125975  . PMID 11953308. doi:10.1093/emboj/21.8.1889.

[Hedley-1] Hedley PL; Jorgensen P; Schlamowitz S; Wangari, Romilda; et al. The genetic basis of long QT and short QT syndromes: a mutation update. Human Mutation. 2009, 30 (11): 1486–511. PMID 19862833. doi:10.1002/humu.21106.

[Sanguinetti_MC,_Tristani-Firouzi_M_2006_463–9-2] Sanguinetti MC, Tristani-Firouzi M. hERG potassium channels and cardiac arrhythmia. Nature. March 2006, 440 (7083): 463–9. PMID 16554806. doi:10.1038/nature04710.

[3] Chiesa N, Rosati B, Arcangeli A, Olivotto M, Wanke E. A novel role for HERG K+ channels: spike-frequency adaptation. J. Physiol. (Lond.). June 1997,. 501 ( Pt 2) (2): 313–8. PMC 1159479  . PMID 9192303. doi:10.1111/j.1469-7793.1997.313bn.x. Overholt JL, Ficker E, Yang T, Shams H, Bright GR, Prabhakar NR. Chemosensing at the carotid body. Involvement of a HERG-like potassium current in glomus cells. Adv. Exp. Med. Biol. 2000, 475: 241–8. PMID 10849664. doi:10.1007/0-306-46825-5_22.

[4] Arcangeli A. Expression and role of hERG channels in cancer cells. Novartis Found. Symp. 2005, 266: 225–32; discussion 232–4. PMID 16050271. doi:10.1002/047002142X.ch17.

[5] Warmke JW, Ganetzky B. A family of potassium channel genes related to eag in Drosophila and mammals. Proc. Natl. Acad. Sci. U.S.A. April 1994, 91 (8): 3438–42 [2015-02-09]. PMC 43592  . PMID 8159766. doi:10.1073/pnas.91.8.3438. （原始內容存檔於2019-10-17）.

[pmid11953308-6] Kagan, Anna; Melman Yonathan F; Krumerman Andrew; McDonald Thomas V. 14-3-3 amplifies and prolongs adrenergic stimulation of HERG K+ channel activity. EMBO J. (England). Apr 2002, 21 (8): 1889–98. ISSN 0261-4189. PMC 125975  . PMID 11953308. doi:10.1093/emboj/21.8.1889.

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