{
  "url": "https://dantelabs.com/traits/ketonuria",
  "trait": {
    "bundleId": "2026.10.17-8666dff2",
    "release": "2026-09-01",
    "slug": "ketonuria",
    "id": "HP:0002919",
    "name": "Ketonuria",
    "definition": "High levels of ketone bodies (acetoacetic acid, beta-hydroxybutyric acid, and acetone) in the urine. Ketone bodies are insignificant in the blood and urine of normal individuals in the postprandial or overnight-fasted state.",
    "synonyms": [
      "Ketonaciduria",
      "Ketone bodies in urine"
    ],
    "systems": [
      {
        "slug": "abnormality-of-metabolism-homeostasis",
        "name": "Laboratory abnormality",
        "url": "https://dantelabs.com/traits/abnormality-of-metabolism-homeostasis"
      },
      {
        "slug": "abnormality-of-the-genitourinary-system",
        "name": "Abnormality of the genitourinary system",
        "url": "https://dantelabs.com/traits/abnormality-of-the-genitourinary-system"
      }
    ],
    "broader": [
      {
        "slug": "abnormal-urine-metabolite-level",
        "name": "Abnormal urine metabolite level",
        "url": "https://dantelabs.com/traits/abnormal-urine-metabolite-level"
      }
    ],
    "narrower": [
      {
        "slug": "elevated-urine-acetoacetic-acid-level",
        "name": "Elevated urine acetoacetic acid level",
        "genes": 2,
        "url": "https://dantelabs.com/traits/elevated-urine-acetoacetic-acid-level"
      }
    ],
    "genes": [
      {
        "slug": "abcc8",
        "symbol": "ABCC8",
        "hgnc": "HGNC:59",
        "name": "ATP binding cassette subfamily C member 8",
        "frequency": "obligate",
        "diseases": 2,
        "url": "https://dantelabs.com/genes/abcc8"
      },
      {
        "slug": "cox14",
        "symbol": "COX14",
        "hgnc": "HGNC:28216",
        "name": "cytochrome c oxidase assembly factor COX14",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/cox14"
      },
      {
        "slug": "cyc1",
        "symbol": "CYC1",
        "hgnc": "HGNC:2579",
        "name": "cytochrome c1",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/cyc1"
      },
      {
        "slug": "cyp27b1",
        "symbol": "CYP27B1",
        "hgnc": "HGNC:2606",
        "name": "cytochrome P450 family 27 subfamily B member 1",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/cyp27b1"
      },
      {
        "slug": "fdx2",
        "symbol": "FDX2",
        "hgnc": "HGNC:30546",
        "name": "ferredoxin 2",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/fdx2"
      },
      {
        "slug": "mcee",
        "symbol": "MCEE",
        "hgnc": "HGNC:16732",
        "name": "methylmalonyl-CoA epimerase",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mcee"
      },
      {
        "slug": "mmaa",
        "symbol": "MMAA",
        "hgnc": "HGNC:18871",
        "name": "metabolism of cobalamin associated A",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mmaa"
      },
      {
        "slug": "pax4",
        "symbol": "PAX4",
        "hgnc": "HGNC:8618",
        "name": "paired box 4",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/pax4"
      },
      {
        "slug": "sdhd",
        "symbol": "SDHD",
        "hgnc": "HGNC:10683",
        "name": "succinate dehydrogenase complex subunit D",
        "frequency": "obligate",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/sdhd"
      },
      {
        "slug": "acat1",
        "symbol": "ACAT1",
        "hgnc": "HGNC:93",
        "name": "acetyl-CoA acetyltransferase 1",
        "frequency": "very-frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/acat1"
      },
      {
        "slug": "tango2",
        "symbol": "TANGO2",
        "hgnc": "HGNC:25439",
        "name": "transport and golgi organization 2 homolog",
        "frequency": "very-frequent",
        "diseases": 2,
        "url": "https://dantelabs.com/genes/tango2"
      },
      {
        "slug": "cox16",
        "symbol": "COX16",
        "hgnc": "HGNC:20213",
        "name": "cytochrome c oxidase assembly factor COX16",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/cox16"
      },
      {
        "slug": "gck",
        "symbol": "GCK",
        "hgnc": "HGNC:4195",
        "name": "glucokinase",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/gck"
      },
      {
        "slug": "gys2",
        "symbol": "GYS2",
        "hgnc": "HGNC:4707",
        "name": "glycogen synthase 2",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/gys2"
      },
      {
        "slug": "hmgcs2",
        "symbol": "HMGCS2",
        "hgnc": "HGNC:5008",
        "name": "3-hydroxy-3-methylglutaryl-CoA synthase 2",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/hmgcs2"
      },
      {
        "slug": "ins",
        "symbol": "INS",
        "hgnc": "HGNC:6081",
        "name": "insulin",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/ins"
      },
      {
        "slug": "ivd",
        "symbol": "IVD",
        "hgnc": "HGNC:6186",
        "name": "isovaleryl-CoA dehydrogenase",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/ivd"
      },
      {
        "slug": "kcnj11",
        "symbol": "KCNJ11",
        "hgnc": "HGNC:6257",
        "name": "potassium inwardly rectifying channel subfamily J member 11",
        "frequency": "frequent",
        "diseases": 2,
        "url": "https://dantelabs.com/genes/kcnj11"
      },
      {
        "slug": "oxct1",
        "symbol": "OXCT1",
        "hgnc": "HGNC:8527",
        "name": "3-oxoacid CoA-transferase 1",
        "frequency": "frequent",
        "diseases": 2,
        "url": "https://dantelabs.com/genes/oxct1"
      },
      {
        "slug": "pdx1",
        "symbol": "PDX1",
        "hgnc": "HGNC:6107",
        "name": "pancreatic and duodenal homeobox 1",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/pdx1"
      },
      {
        "slug": "slc16a1",
        "symbol": "SLC16A1",
        "hgnc": "HGNC:10922",
        "name": "solute carrier family 16 member 1",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/slc16a1"
      },
      {
        "slug": "slc2a2",
        "symbol": "SLC2A2",
        "hgnc": "HGNC:11006",
        "name": "solute carrier family 2 member 2",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/slc2a2"
      },
      {
        "slug": "stat3",
        "symbol": "STAT3",
        "hgnc": "HGNC:11364",
        "name": "signal transducer and activator of transcription 3",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/stat3"
      },
      {
        "slug": "tamm41",
        "symbol": "TAMM41",
        "hgnc": "HGNC:25187",
        "name": "TAM41 mitochondrial translocator assembly and maintenance homolog",
        "frequency": "frequent",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/tamm41"
      },
      {
        "slug": "hmgcl",
        "symbol": "HMGCL",
        "hgnc": "HGNC:5005",
        "name": "3-hydroxy-3-methylglutaryl-CoA lyase",
        "frequency": "occasional",
        "diseases": 2,
        "url": "https://dantelabs.com/genes/hmgcl"
      },
      {
        "slug": "lrpprc",
        "symbol": "LRPPRC",
        "hgnc": "HGNC:15714",
        "name": "leucine rich pentatricopeptide repeat containing",
        "frequency": "occasional",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/lrpprc"
      },
      {
        "slug": "mmachc",
        "symbol": "MMACHC",
        "hgnc": "HGNC:24525",
        "name": "metabolism of cobalamin associated C",
        "frequency": "occasional",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mmachc"
      },
      {
        "slug": "mto1",
        "symbol": "MTO1",
        "hgnc": "HGNC:19261",
        "name": "mitochondrial tRNA translation optimization 1",
        "frequency": "occasional",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mto1"
      },
      {
        "slug": "slc25a13",
        "symbol": "SLC25A13",
        "hgnc": "HGNC:10983",
        "name": "solute carrier family 25 member 13",
        "frequency": "occasional",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/slc25a13"
      },
      {
        "slug": "sugct",
        "symbol": "SUGCT",
        "hgnc": "HGNC:16001",
        "name": "succinyl-CoA:glutarate-CoA transferase",
        "frequency": "occasional",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/sugct"
      },
      {
        "slug": "ca5a",
        "symbol": "CA5A",
        "hgnc": "HGNC:1377",
        "name": "carbonic anhydrase 5A",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/ca5a"
      },
      {
        "slug": "gcdh",
        "symbol": "GCDH",
        "hgnc": "HGNC:4189",
        "name": "glutaryl-CoA dehydrogenase",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/gcdh"
      },
      {
        "slug": "mccc1",
        "symbol": "MCCC1",
        "hgnc": "HGNC:6936",
        "name": "methylcrotonyl-CoA carboxylase subunit 1",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mccc1"
      },
      {
        "slug": "mccc2",
        "symbol": "MCCC2",
        "hgnc": "HGNC:6937",
        "name": "methylcrotonyl-CoA carboxylase subunit 2",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mccc2"
      },
      {
        "slug": "mmab",
        "symbol": "MMAB",
        "hgnc": "HGNC:19331",
        "name": "metabolism of cobalamin associated B",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/mmab"
      },
      {
        "slug": "pck1",
        "symbol": "PCK1",
        "hgnc": "HGNC:8724",
        "name": "phosphoenolpyruvate carboxykinase 1",
        "diseases": 1,
        "url": "https://dantelabs.com/genes/pck1"
      }
    ]
  }
}