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Gene and Protein Information  |
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| Species | TM | P Loops | AA | Chromosomal Location | Gene Symbol | Gene Name | Reference |
| Human | 2 | 1 | 360 | 2q37.1 | KCNJ13 | potassium inwardly rectifying channel subfamily J member 13 | 2,5,10 |
| Mouse | 2 | 0 | 360 | 1 | Kcnj13 | potassium inwardly-rectifying channel, subfamily J, member 13 | |
| Rat | 2 | 1 | 360 | 9q35 | Kcnj13 | potassium inwardly-rectifying channel, subfamily J, member 13 | 3 |
| Previous and Unofficial Names |
| potassium inwardly rectifying channel subfamily J member 13 | inward rectifier K(+) channel Kir7.1 | inward rectifier potassium channel 13 | IRK13 | Kir1.4 | potassium channel, inwardly rectifying subfamily J, member 13 | potassium inwardly-rectifying channel | potassium voltage-gated channel subfamily J member 13 |
| Database Links |
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| Alphafold | O60928 (Hs), P86046 (Mm), O70617 (Rn) |
| CATH/Gene3D | 2.60.40.1400 |
| ChEMBL Target | CHEMBL2146349 (Hs) |
| Ensembl Gene | ENSG00000115474 (Hs), ENSMUSG00000079436 (Mm), ENSRNOG00000016057 (Rn) |
| Entrez Gene | 3769 (Hs), 100040591 (Mm), 94341 (Rn) |
| Human Protein Atlas | ENSG00000115474 (Hs) |
| KEGG Gene | hsa:3769 (Hs), mmu:100040591 (Mm), rno:94341 (Rn) |
| OMIM | 603208 (Hs) |
| Orphanet | ORPHA159740 (Hs) |
| Pharos | O60928 (Hs) |
| RefSeq Nucleotide | NM_002242 (Hs), NM_001110227 (Mm), NM_053608 (Rn) |
| RefSeq Protein | NP_002233 (Hs), NP_001103697 (Mm), NP_446060 (Rn) |
| UniProtKB | O60928 (Hs), P86046 (Mm), O70617 (Rn) |
| Wikipedia | KCNJ13 (Hs) |
| Functional Characteristics |
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| Inward-rectifier current | |
| Ion Selectivity and Conductance |
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| Voltage Dependence |
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| Voltage Dependence Comments | ||||||||||||||||
| Kir7.1 is voltage dependant, however unlike other Kir channels shows no crossover phenomenon (increasing in outward current as [K+o] increases) [5]. |
Download all structure-activity data for this target as a CSV file 
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| Key to terms and symbols | View all chemical structures | Click column headers to sort | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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| Channel Blocker Comments | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| ML-111 blocks human Kir7.1 channel activity by >50% at 10 micromolar concentration [1]. VU573 blocks human Kir7.1 channel activity by >50% at 5 micromolar concentration [11]. |
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| Tissue Distribution
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| Functional Assays
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| Physiological Functions
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| Clinically-Relevant Mutations and Pathophysiology
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| Biologically Significant Variant Comments |
| Three major, and many minor, variants of rat Kir7.1 mRNA have been found to arise from alternative splicing of non-coding exons in the 5' untranslated region. Tissue-specific variation in splicing patterns is widespread [7]. |
1. Denton JS, Weaver CD, Lewis LM, Chauder BA, Lindsley CW. (2010) Discovery of a small molecule inhibitor of ROMK and Kir7.1. In Probe Reports from the NIH Molecular Libraries Program. (National Center for Biotechnology Information (US)) . [PMID:21433378]
2. Derst C, Döring F, Preisig-Müller R, Daut J, Karschin A, Jeck N, Weber S, Engel H, Grzeschik KH. (1998) Partial gene structure and assignment to chromosome 2q37 of the human inwardly rectifying K+ channel (Kir7.1) gene (KCNJ13). Genomics, 54 (3): 560-3. [PMID:9878260]
3. Döring F, Derst C, Wischmeyer E, Karschin C, Schneggenburger R, Daut J, Karschin A. (1998) The epithelial inward rectifier channel Kir7.1 displays unusual K+ permeation properties. J Neurosci, 18 (21): 8625-36. [PMID:9786970]
4. Hejtmancik JF, Jiao X, Li A, Sergeev YV, Ding X, Sharma AK, Chan CC, Medina I, Edwards AO. (2008) Mutations in KCNJ13 cause autosomal-dominant snowflake vitreoretinal degeneration. Am J Hum Genet, 82 (1): 174-80. [PMID:18179896]
5. Krapivinsky G, Medina I, Eng L, Krapivinsky L, Yang Y, Clapham DE. (1998) A novel inward rectifier K+ channel with unique pore properties. Neuron, 20 (5): 995-1005. [PMID:9620703]
6. Kusaka S, Inanobe A, Fujita A, Makino Y, Tanemoto M, Matsushita K, Tano Y, Kurachi Y. (2001) Functional Kir7.1 channels localized at the root of apical processes in rat retinal pigment epithelium. J Physiol (Lond.), 531 (Pt 1): 27-36. [PMID:11179389]
7. Nakamura N, Suzuki Y, Ikeda Y, Notoya M, Hirose S. (2000) Complex structure and regulation of expression of the rat gene for inward rectifier potassium channel Kir7.1. J Biol Chem, 275 (36): 28276-84. [PMID:10871613]
8. Nakamura N, Suzuki Y, Sakuta H, Ookata K, Kawahara K, Hirose S. (1999) Inwardly rectifying K+ channel Kir7.1 is highly expressed in thyroid follicular cells, intestinal epithelial cells and choroid plexus epithelial cells: implication for a functional coupling with Na+,K+-ATPase. Biochem J, 342 ( Pt 2): 329-36. [PMID:10455019]
9. Ookata K, Tojo A, Suzuki Y, Nakamura N, Kimura K, Wilcox CS, Hirose S. (2000) Localization of inward rectifier potassium channel Kir7.1 in the basolateral membrane of distal nephron and collecting duct. J Am Soc Nephrol, 11 (11): 1987-94. [PMID:11053473]
10. Partiseti M, Collura V, Agnel M, Culouscou JM, Graham D. (1998) Cloning and characterization of a novel human inwardly rectifying potassium channel predominantly expressed in small intestine. FEBS Lett, 434 (1-2): 171-6. [PMID:9738472]
11. Raphemot R, Lonergan DF, Nguyen TT, Utley T, Lewis LM, Kadakia R, Weaver CD, Gogliotti R, Hopkins C, Lindsley CW et al.. (2011) Discovery, characterization, and structure-activity relationships of an inhibitor of inward rectifier potassium (Kir) channels with preference for Kir2.3, Kir3.x, and Kir7.1. Front Pharmacol, 2: 75. [PMID:22275899]
12. Sergouniotis PI, Davidson AE, Mackay DS, Li Z, Yang X, Plagnol V, Moore AT, Webster AR. (2011) Recessive mutations in KCNJ13, encoding an inwardly rectifying potassium channel subunit, cause leber congenital amaurosis. Am J Hum Genet, 89 (1): 183-90. [PMID:21763485]
13. Shimura M, Yuan Y, Chang JT, Zhang S, Campochiaro PA, Zack DJ, Hughes BA. (2001) Expression and permeation properties of the K(+) channel Kir7.1 in the retinal pigment epithelium. J Physiol (Lond.), 531 (Pt 2): 329-46. [PMID:11230507]
14. Suzuki Y, Yasuoka Y, Shimohama T, Nishikitani M, Nakamura N, Hirose S, Kawahara K. (2003) Expression of the K+ channel Kir7.1 in the developing rat kidney: role in K+ excretion. Kidney Int, 63 (3): 969-75. [PMID:12631077]
15. Yang D, Zhang X, Hughes BA. (2008) Expression of inwardly rectifying potassium channel subunits in native human retinal pigment epithelium. Exp Eye Res, 87 (3): 176-83. [PMID:18653180]
16. Yasuda K, Shimura M, Nakazawa T, Sato H, Tomita H, Sugano E, Tamai M. (2003) Expression and functional properties of unique inward rectifier K+ channel Kir7.1 in the porcine iris and retinal pigment epithelium. Curr Eye Res, 27 (5): 279-87. [PMID:14562164]
17. Zhang W, Zhang X, Wang H, Sharma AK, Edwards AO, Hughes BA. (2013) Characterization of the R162W Kir7.1 mutation associated with snowflake vitreoretinopathy. Am J Physiol, Cell Physiol, 304 (5): C440-9. [PMID:23255580]
