WordNet
- abnormal presence of amino acids in the urine; usually a symptom of metabolic defects
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/07/08 16:20:50」(JST)
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| Aminoaciduria |
| Classification and external resources |
| DiseasesDB |
14901 |
| MedlinePlus |
003366 |
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[edit on Wikidata]
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Aminoaciduria is the presence of amino acids in the urine. Small amounts of amino acids are also present in normal urine. Increased total urine amino acids may result from metabolic disorders, chronic liver disease or renal disorders. Aminoacidurias can be divided into primary and secondary aminoacidurias.
[1]==See also==
- List of amino acid metabolism disorders
‹ The template below (Abnormal clinical and laboratory findings for urine) is being merged. See templates for discussion for the discussion that led to this result. ›
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Components and results of urine tests (CPT 81000–81099; R80–R82, 791)
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| Components |
- Albumin
- Myoglobin
- hCG
- Leukocyte esterase
- Urine pregnancy test
- Ketone bodies
- Glucose
- Urobilinogen
- Bilirubin
- Creatinine
- RBC
- WBC
- Urinary casts
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| Chemical properties |
- Urine specific gravity
- Urine osmolality
- Urine pH
- Urine anion gap
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| Abnormal findings |
| Red blood cells |
- Hematuria (Microscopic hematuria)
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| White blood cells |
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| Proteinuria |
- Albuminuria/Microalbuminuria
- Myoglobinuria
- Hemoglobinuria
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| Small molecules |
- Glycosuria
- Ketonuria
- Bilirubinuria
- Hyperuricosuria/Hypouricosuria
- Aminoaciduria
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| Pathogens |
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| Other |
- Chyluria
- Crystalluria
- osmolality (Isosthenuria, Hypersthenuria)
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Inborn error of amino acid metabolism (E70–E72, 270)
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| K→acetyl-CoA |
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Lysine/straight chain
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- Glutaric acidemia type 1
- type 2
- Hyperlysinemia
- Pipecolic acidemia
- Saccharopinuria
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Leucine
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- 3-hydroxy-3-methylglutaryl-CoA lyase deficiency
- 3-Methylcrotonyl-CoA carboxylase deficiency
- 3-Methylglutaconic aciduria 1
- Isovaleric acidemia
- Maple syrup urine disease
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Tryptophan
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| G |
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G→pyruvate→citrate
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Glycine
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- D-Glyceric acidemia
- Glutathione synthetase deficiency
- Sarcosinemia
- Glycine→Creatine: GAMT deficiency
- Glycine encephalopathy
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G→glutamate→
α-ketoglutarate
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Histidine
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- Carnosinemia
- Histidinemia
- Urocanic aciduria
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Proline
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- Hyperprolinemia
- Prolidase deficiency
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Glutamate/glutamine
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G→propionyl-CoA→
succinyl-CoA
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Valine
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- Hypervalinemia
- Isobutyryl-CoA dehydrogenase deficiency
- Maple syrup urine disease
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Isoleucine
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- 2-Methylbutyryl-CoA dehydrogenase deficiency
- Beta-ketothiolase deficiency
- Maple syrup urine disease
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Methionine
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- Cystathioninuria
- Homocystinuria
- Hypermethioninemia
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General BC/OA
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- Methylmalonic acidemia
- Methylmalonyl-CoA mutase deficiency
- Propionic acidemia
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G→fumarate
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Phenylalanine/tyrosine
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Phenylketonuria
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- 6-Pyruvoyltetrahydropterin synthase deficiency
- Tetrahydrobiopterin deficiency
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Tyrosinemia
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- Alkaptonuria/Ochronosis
- Type I tyrosinemia
- Type II tyrosinemia
- Type III tyrosinemia/Hawkinsinuria
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Tyrosine→Melanin
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- Albinism: Ocular albinism (1)
- Oculocutaneous albinism (Hermansky–Pudlak syndrome)
- Waardenburg syndrome
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Tyrosine→Norepinephrine
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- Dopamine beta hydroxylase deficiency
- reverse: Brunner syndrome
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G→oxaloacetate
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Urea cycle/Hyperammonemia
(arginine
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- Argininemia
- Argininosuccinic aciduria
- Carbamoyl phosphate synthetase I deficiency
- Citrullinemia
- N-Acetylglutamate synthase deficiency
- Ornithine transcarbamylase deficiency/translocase deficiency
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Transport/
IE of RTT |
- Solute carrier family: Cystinuria
- Hartnup disease
- Iminoglycinuria
- Lysinuric protein intolerance
- Fanconi syndrome: Oculocerebrorenal syndrome
- Cystinosis
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| Other |
- 2-Hydroxyglutaric aciduria
- Aminoacylase 1 deficiency
- Ethylmalonic encephalopathy
- Fumarase deficiency
- Trimethylaminuria
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References
- ^ "Aminoacidurea and its classification". All Medical Stuff.
UpToDate Contents
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English Journal
- T-type amino acid transporter TAT1 (Slc16a10) is essential for extracellular aromatic amino acid homeostasis control.
- Mariotta L, Ramadan T, Singer D, Guetg A, Herzog B, Stoeger C, Palacín M, Lahoutte T, Camargo SM, Verrey F.SourceInstitute of Physiology and Zurich Center for Integrative Human Physiology, University of Zurich;
- The Journal of physiology.J Physiol.2012 Nov 12. [Epub ahead of print]
- The uniporter TAT1 (Slc16a10) mediates the facilitated diffusion of aromatic amino acids across basolateral membranes of kidney, small intestine and liver epithelial cells and across the plasma membrane of non-epithelial cells like skeletal myocytes. Its role for body amino acid homeostasis was now
- PMID 23045339
- Lysine triggers apoptosis through a NADPH oxidase-dependent mechanism in human renal tubular cells.
- Verzola D, Famà A, Villaggio B, Di Rocco M, Simonato A, D'Amato E, Gianiorio F, Garibotto G.SourceDepartment of Internal Medicine, Nephrology Division, Genoa University, IRCSS Azienda Ospedaliera Universitaria San Martino - IST, Viale Benedetto XV,6, 16132, Genoa, Italy.
- Journal of inherited metabolic disease.J Inherit Metab Dis.2012 Nov;35(6):1011-9. doi: 10.1007/s10545-012-9468-z. Epub 2012 Mar 9.
- Progressive chronic kidney disease (CKD) is common in lysinuric protein intolerance (LPI), a primary inherited aminoaciduria characterized by massive Lysine excretion in urine. However, by which mechanisms Lysine may cause kidney damage to tubule cells is still not understood. This study determined
- PMID 22403019
Japanese Journal
- Acquired Fanconi syndrome in a dog exposed to jerky treats in Japan
- Acquired Fanconi syndrome in a dog exposed to jerky treats in Japan
Related Links
- Aminoaciduria is the presence of amino acids in the urine. Small amounts of amino acids are also present in normal urine. Increased total urine amino acids may result from metabolic disorders, chronic liver disease or renal disorders.
Related Pictures
★リンクテーブル★
[★]
- 英
- aminoaciduria
- 関
- アミノ酸代謝異常症
[★]
高二塩基性アミノ酸尿症II型
[★]
二塩基性アミノ酸尿症I型
[★]
高二塩基性アミノ酸尿症