{
  "meta": {
    "disclaimer": "Do not rely on openFDA to make decisions regarding medical care. While we make every effort to ensure that data is accurate, you should assume all results are unvalidated. We may limit or otherwise restrict your access to the API in line with our Terms of Service.",
    "terms": "https://open.fda.gov/terms/",
    "license": "https://open.fda.gov/license/",
    "last_updated": "2026-09-09",
    "results": {
      "skip": 0,
      "limit": 1,
      "total": 1
    }
  },
  "results": [
    {
      "codes": [
        {
          "uuid": "5fa343b3-52c0-4ca4-9820-69da60d90e54",
          "code": "P37088",
          "type": "PRIMARY",
          "url": "https://www.uniprot.org/uniprot/P37088",
          "code_system": "UNIPROT"
        },
        {
          "uuid": "26806fc3-8872-4f5f-b0d3-40cfc757f171",
          "code": "P51168",
          "type": "PRIMARY",
          "url": "https://www.uniprot.org/uniprot/P51168",
          "code_system": "UNIPROT"
        },
        {
          "uuid": "b071367c-e369-49ee-8e7f-5cf0f7250f7e",
          "code": "P51170",
          "type": "PRIMARY",
          "url": "https://www.uniprot.org/uniprot/P51170",
          "code_system": "UNIPROT"
        },
        {
          "uuid": "c5d815ee-a2a8-4024-8ac8-2bd05cb3bc77",
          "code": "ZEX6X6B5QP",
          "type": "PRIMARY",
          "code_system": "FDA UNII"
        }
      ],
      "substance_class": "protein",
      "references": [
        {
          "uuid": "60b4f568-8ea8-25f0-41d0-79d223fa34e0",
          "citation": "https://en.wikipedia.org/wiki/Epithelial_sodium_channel",
          "url": "https://en.wikipedia.org/wiki/Epithelial_sodium_channel",
          "doc_type": "WIKI",
          "public_domain": true,
          "tags": [
            "NOMEN",
            "PUBLIC_DOMAIN_RELEASE"
          ]
        },
        {
          "uuid": "b617803e-1154-c4cd-ff2b-f24ca81b5db6",
          "citation": "https://www.uniprot.org/uniprot/P37088",
          "doc_type": "UNIPROT",
          "public_domain": true,
          "tags": [
            "PUBLIC_DOMAIN_RELEASE"
          ]
        },
        {
          "uuid": "d507bf65-8a75-42e1-8324-b07d8c118c6d",
          "citation": "Nickolaus, P., P. Iacono, and S. Constant. \"ePS1. 07 A single application of the epithelial sodium channel inhibitor BI 1265162 significantly improves water transport and mucociliary clearance of cystic fibrosis epithelial tissue, alone or combined with lumacaftor/ivacaftor or isoproterenol.\" Journal of Cystic Fibrosis 18 (2019): S41.",
          "url": "https://www.cysticfibrosisjournal.com/article/S1569-1993(19)30246-2/abstract",
          "doc_type": "MEETING ABSTRACT",
          "public_domain": true,
          "tags": [
            "POT"
          ]
        },
        {
          "uuid": "068c73c7-cd33-4fd2-80b7-99fcb71786ce",
          "citation": "Contarini, Martina, et al. \"Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis.\" Multidisciplinary respiratory medicine 13.1 (2018): 26.",
          "url": "https://link.springer.com/article/10.1186/s40248-018-0143-6",
          "doc_type": "JA",
          "public_domain": true
        },
        {
          "uuid": "e851cdb4-a8b5-458b-8d99-b21c325d4954",
          "citation": "Contarini, Martina, et al. \"Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis.\" Multidisciplinary respiratory medicine 13.1 (2018): 26.",
          "url": "https://link.springer.com/article/10.1186/s40248-018-0143-6",
          "doc_type": "JA",
          "public_domain": true
        },
        {
          "uuid": "ef2eeb6e-5c3a-421f-9865-ebff7c782e6a",
          "citation": "http://www.probechem.com/target_SodiumChannel-6.html",
          "url": "http://www.probechem.com/target_SodiumChannel-6.html",
          "doc_type": "WEBSITE",
          "public_domain": true,
          "tags": [
            "PUBLIC_DOMAIN_RELEASE"
          ]
        },
        {
          "uuid": "6d3b7e6e-970c-4336-a2c0-4230de75c8bf",
          "citation": "Scott, David W., et al. \"SPX-101 is a novel epithelial sodium channel–targeted therapeutic for cystic fibrosis that restores mucus transport.\" American journal of respiratory and critical care medicine 196.6 (2017): 734-744.",
          "url": "https://www.atsjournals.org/doi/pdf/10.1164/rccm.201612-2445OC",
          "doc_type": "JA",
          "public_domain": true,
          "tags": [
            "PUBLIC_DOMAIN_RELEASE"
          ]
        },
        {
          "uuid": "6ed32c2e-c2c0-47b8-b943-2e7565f3573b",
          "citation": "Scott, David W., et al. \"SPX-101 is a novel epithelial sodium channel–targeted therapeutic for cystic fibrosis that restores mucus transport.\" American journal of respiratory and critical care medicine 196.6 (2017): 734-744.",
          "url": "https://www.atsjournals.org/doi/pdf/10.1164/rccm.201612-2445OC",
          "doc_type": "JA",
          "public_domain": true,
          "tags": [
            "PUBLIC_DOMAIN_RELEASE"
          ]
        }
      ],
      "definition_type": "PRIMARY",
      "definition_level": "COMPLETE",
      "uuid": "9b54ccdd-bb36-4473-a3ae-b78b69a243a6",
      "version": "12",
      "unii": "ZEX6X6B5QP",
      "relationships": [
        {
          "uuid": "fca705e9-b932-4a75-86ec-e52936b42a66",
          "amount": {
            "uuid": "0a9ba4f5-68f1-43aa-866f-b1c671cd4d1d"
          },
          "type": "INHIBITOR->TARGET",
          "references": [
            "ef2eeb6e-5c3a-421f-9865-ebff7c782e6a"
          ],
          "related_substance": {
            "uuid": "701c9a7e-5784-4152-9b38-f5699025d8c3",
            "refuuid": "0430d326-5b94-48ab-99d3-4c504ff4adfa",
            "name": "VOLULORIDE",
            "unii": "K33H7H4188",
            "linking_id": "K33H7H4188",
            "ref_pname": "VOLULORIDE",
            "substance_class": "reference"
          }
        },
        {
          "uuid": "bd9e24b9-fd64-4426-81b5-5372f8ecec3c",
          "amount": {
            "uuid": "e3358af4-8c3a-4071-be06-185a861130ab"
          },
          "type": "INHIBITOR->TARGET",
          "references": [
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          ],
          "related_substance": {
            "uuid": "21200e6b-ddc7-4a68-81ab-c1c96e1eac47",
            "refuuid": "56be91cc-e4bf-4380-bc60-e51b853f3861",
            "name": "IDREVLORIDE",
            "unii": "NY2J56S3YA",
            "linking_id": "NY2J56S3YA",
            "ref_pname": "IDREVLORIDE"
          }
        },
        {
          "uuid": "f28f5e01-1d35-4fae-956d-a4d9af099c9e",
          "amount": {
            "uuid": "11c0a5b5-27ff-4a92-bd71-4edc8e7f40b2"
          },
          "type": "INHIBITOR->TARGET",
          "references": [
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          ],
          "related_substance": {
            "uuid": "163c1b5e-09a4-40c8-a665-a03c73342149",
            "refuuid": "6471bc83-0c8a-469c-945a-cd5b9cadbd24",
            "name": "BI-1265162",
            "unii": "F160Q9V54P",
            "linking_id": "F160Q9V54P",
            "ref_pname": "BI-1265162"
          }
        },
        {
          "uuid": "9558f742-bf30-49ca-a753-3453eb243dc2",
          "amount": {
            "uuid": "0bf1b3d8-ada6-40de-997c-915a26392968",
            "average": 261,
            "units": "NANOMOLAR"
          },
          "comments": "Binds selectively to ENaC and promotes internalization of the a-, b-, and g-subunits.\nRemoving ENaC from the membrane with SPX-101 causes a significant decrease in amiloride-sensitive current.",
          "qualification": "EC50",
          "type": "INHIBITOR->TARGET",
          "references": [
            "6d3b7e6e-970c-4336-a2c0-4230de75c8bf"
          ],
          "related_substance": {
            "uuid": "69a23bc5-8c16-4d09-834a-8b03f845f0f6",
            "refuuid": "3fa932d4-173c-4d4e-93c5-f2d01359cb9e",
            "name": "SPX-101",
            "unii": "A6KAB92LQ7",
            "linking_id": "A6KAB92LQ7",
            "ref_pname": "SPX-101",
            "substance_class": "reference"
          }
        }
      ],
      "names": [
        {
          "uuid": "a8e95bc2-623f-967e-72f6-e2d8d70f173c",
          "name": "AMILORIDE-SENSITIVE SODIUM CHANNEL",
          "stdName": "AMILORIDE-SENSITIVE SODIUM CHANNEL",
          "type": "cn",
          "languages": [
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          ],
          "preferred": false,
          "references": [
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          ],
          "display_name": true
        },
        {
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          "name": "ENAC",
          "stdName": "ENAC",
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          "languages": [
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          ],
          "preferred": false,
          "references": [
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          ],
          "display_name": false
        },
        {
          "uuid": "f176feba-edb6-434b-96bb-141ce5329f07",
          "name": "Epithelial Na(+) channel",
          "stdName": "EPITHELIAL NA(+) CHANNEL",
          "type": "cn",
          "languages": [
            "en"
          ],
          "preferred": false,
          "references": [
            "b617803e-1154-c4cd-ff2b-f24ca81b5db6"
          ],
          "display_name": false
        },
        {
          "uuid": "4ceeae48-18ca-4b9c-82c5-4328bf2ef4f8",
          "name": "Epithelial Sodium Channel–",
          "stdName": "EPITHELIAL SODIUM CHANNEL-",
          "type": "cn",
          "languages": [
            "en"
          ],
          "preferred": false,
          "references": [
            "60b4f568-8ea8-25f0-41d0-79d223fa34e0"
          ],
          "display_name": false
        },
        {
          "uuid": "f66af173-9d0f-43ee-8baf-bcd6e01b8368",
          "name": "Nonvoltage-gated sodium channel 1",
          "stdName": "NONVOLTAGE-GATED SODIUM CHANNEL 1",
          "type": "cn",
          "languages": [
            "en"
          ],
          "preferred": false,
          "references": [
            "b617803e-1154-c4cd-ff2b-f24ca81b5db6"
          ],
          "display_name": false
        }
      ],
      "protein": {
        "uuid": "fa6e2331-ecbe-4f0b-9be1-fbbfa779d2b1",
        "glycosylation": {
          "uuid": "2e14902f-4da5-48d0-a3a2-61f579e14c3d",
          "glycosylation_type": "HUMAN",
          "n_glycosylation_sites": [
            {
              "residue_index": 260,
              "subunit_index": 2
            },
            {
              "residue_index": 209,
              "subunit_index": 3
            },
            {
              "residue_index": 497,
              "subunit_index": 3
            }
          ]
        },
        "subunits": [
          {
            "uuid": "98d6c673-84de-49c5-a6af-3f68e1203f34",
            "sequence": "MEGNKLEEQDSSPPQSTPGLMKGNKREEQGLGPEPAAPQQPTAEEEALIEFHRSYRELFEFFCNNTTIHGAIRLVCSQHNRMKTAFWAVLWLCTFGMMYWQFGLLFGEYFSYPVSLNINLNSDKLVFPAVTICTLNPYRYPEIKEELEELDRITEQTLFDLYKYSSFTTLVAGSRSRRDLRGTLPHPLQRLRVPPPPHGARRARSVASSLRDNNPQVDWKDWKIGFQLCNQNKSDCFYQTYSSGVDAVREWYRFHYINILSRLPETLPSLEEDTLGNFIFACRFNQVSCNQANYSHFHHPMYGNCYTFNDKNNSNLWMSSMPGINNGLSLMLRAEQNDFIPLLSTVTGARVMVHGQDEPAFMDDGGFNLRPGVETSISMRKETLDRLGGDYGDCTKNGSDVPVENLYPSKYTQQVCIHSCFQESMIKECGCAYIFYPRPQNVEYCDYRKHSSWGYCYYKLQVDFSSDHLGCFTKCRKPCSVTSYQLSAGYSRWPSVTSQEWVFQMLSRQNNYTVNNKRNGVAKVNIFFKELNYKTNSESPSVTMVTLLSNLGSQWSLWFGSSVLSVVEMAELVFDLLVIMFLMLLRRFRSRYWSPGRGGRGAQEVASTLASSPPSHFCPHPMSLSLSQPGPAPSPALTAPPPAYATLGPRPSPGGSAGASSSTCPLGGP",
            "subunit_index": 1
          },
          {
            "uuid": "fa6c5c62-5fb1-4580-bdad-1d3128ac6d52",
            "sequence": "MHVKKYLLKGLHRLQKGPGYTYKELLVWYCDNTNTHGPKRIICEGPKKKAMWFLLTLLFAALVCWQWGIFIRTYLSWEVSVSLSVGFKTMDFPAVTICNASPFKYSKIKHLLKDLDELMEAVLERILAPELSHANATRNLNFSIWNHTPLVLIDERNPHHPMVLDLFGDNHNGLTSSSASEKICNAHGCKMAMRLCSLNRTQCTFRNFTSATQALTEWYILQATNIFAQVPQQELVEMSYPGEQMILACLFGAEPCNYRNFTSIFYPHYGNCYIFNWGMTEKALPSANPGTEFGLKLILDIGQEDYVPFLASTAGVRLMLHEQRSYPFIRDEGIYAMSGTETSIGVLVDKLQRMGEPYSPCTVNGSEVPVQNFYSDYNTTYSIQACLRSCFQDHMIRNCNCGHYLYPLPRGEKYCNNRDFPDWAHCYSDLQMSVAQRETCIGMCKESCNDTQYKMTISMADWPSEASEDWIFHVLSQERDQSTNITLSRKGIVKLNIYFQEFNYRTIEESAANNIVWLLSNLGGQFGFWMGGSVLCLIEFGEIIIDFVWITIIKLVALAKSLRQRRAQASYAGPPPTVAELVEAHTNFGFQPDTAPRSPNTGPYPSEQALPIPGTPPPNYDSLRLQPLDVIESDSEGDAI",
            "subunit_index": 2
          },
          {
            "uuid": "8d81ae8f-ee90-4e8b-b5ba-0a0a5db1f56e",
            "sequence": "MAPGEKIKAKIKKNLPVTGPQAPTIKELMRWYCLNTNTHGCRRIVVSRGRLRRLLWIGFTLTAVALILWQCALLVFSFYTVSVSIKVHFRKLDFPAVTICNINPYKYSTVRHLLADLEQETREALKSLYGFPESRKRREAESWNSVSEGKQPRFSHRIPLLIFDQDEKGKARDFFTGRKRKVGGSIIHKASNVMHIESKQVVGFQLCSNDTSDCATYTFSSGINAIQEWYKLHYMNIMAQVPLEKKINMSYSAEELLVTCFFDGVSCDARNFTLFHHPMHGNCYTFNNRENETILSTSMGGSEYGLQVILYINEEEYNPFLVSSTGAKVIIHRQDEYPFVEDVGTEIETAMVTSIGMHLTESFKLSEPYSQCTEDGSDVPIRNIYNAAYSLQICLHSCFQTKMVEKCGCAQYSQPLPPAANYCNYQQHPNWMYCYYQLHRAFVQEELGCQSVCKEACSFKEWTLTTSLAQWPSVVSEKWLLPVLTWDQGRQVNKKLNKTDLAKLLIFYKDLNQRSIMESPANSIEMLLSNFGGQLGLWMSCSVVCVIEIIEVFFIDFFSIIARRQWQKAKEWWAWKQAPPCPEAPRSPQGQDNPALDIDDDLPTFNSALHLPPALGTQVPGTPPPKYNTLRLERAFSNQLTDTQMLDEL",
            "subunit_index": 3
          }
        ],
        "references": [
          "b617803e-1154-c4cd-ff2b-f24ca81b5db6",
          "60b4f568-8ea8-25f0-41d0-79d223fa34e0"
        ],
        "protein_sub_type": "ION CHANNEL",
        "sequence_origin": "HUMAN",
        "sequence_type": "COMPLETE"
      },
      "properties": [
        {
          "uuid": "7e9fcfe9-bfcd-444b-987f-c8cdc99cb444",
          "name": "MOL_WEIGHT:NUMBER(CALCULATED)",
          "type": "amount",
          "value": {
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            "type": "ESTIMATED",
            "average": 223000,
            "units": "Da"
          },
          "defining": false,
          "property_type": "CHEMICAL"
        },
        {
          "uuid": "fc347aaa-c2de-4f30-911c-5d46759c5430",
          "name": "Molecular Formula",
          "value": {
            "uuid": "e828c4e0-42cc-49cc-8ce2-0ec6fb57bf31",
            "type": "ESTIMATED",
            "non_numeric_value": "C9972H15249N2667O2895S119"
          },
          "defining": false,
          "property_type": "CHEMICAL"
        }
      ],
      "modifications": {
        "uuid": "aaa7211e-5f47-4821-b10c-19467a96ea17",
        "structural_modifications": [
          {
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            "sites": [
              {
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                "subunit_index": 2
              },
              {
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                "subunit_index": 2
              }
            ],
            "location_type": "SITE_SPECIFIC",
            "molecular_fragment": {
              "uuid": "109ffc2e-e566-4259-bd35-6796237ffa79",
              "refuuid": "b6eb2edb-1b4d-46ad-92ad-a43f0f545926",
              "name": "DEXFOSFOSERINE",
              "unii": "VI4F0K069V",
              "linking_id": "VI4F0K069V",
              "ref_pname": "DEXFOSFOSERINE",
              "substance_class": "reference"
            },
            "structural_modification_type": "AMINO_ACID_SUBSTITUTION"
          }
        ]
      }
    }
  ]
}