Dihydrouracil

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Dihydrouracil
2D structure for Dihydrouracil
Chemical Name 1,3-diazinane-2,4-dione
Chemical Formula C4H6N2O2
CAS Number 504-07-4
Chemical Information HMDB00076
Biochemical Taxonomy

  • Pyrimidines and Pyrimidine Derivatives

Functional Taxonomy Not Available
Nutritional Taxonomy Not Available
Metabolic Pathways

  • Pantothenate and CoA Biosynthesis

Biofluid Location

  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine

Tissue Location

  • Kidney
  • Liver
  • Fibroblasts

Normal Biofluid Concentrations

  • Blood: 3.33 +/- 7.07 uM
  • Cerebrospinal Fluid (CSF): 2.1 +/- 1.0 uM
  • Urine: 0.79 umol/mmol creatinine
  • Urine: 12.2 +/- 6.2 umol/mmol creatinine

Normal Tissue Concentrations Not Available
Diseases / Conditions Related to Nutrition

  • Dihydropyrimidine dehydrogenase (DPD) deficiency

Other (Monogenic Disorders) Not Available
Abnormal Biofluid Concentrations

  • Cerebrospinal Fluid (CSF) (Dihydropyrimidine dehydrogenase (DPD) deficiency): 81.5 (46.0-117.0) uM
  • Urine (Dihydropyrimidine dehydrogenase (DPD) deficiency): 627.0 umol/mmol creatinine

Abnormal Tissue Concentrations Not Available
Physiological Processes Not Available
Authors:
Affiliations:

Contents

Introduction

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An intermediate breakdown product of uracil. Dihydropyrimidine dehydrogenase catalyzes the reduction of uracil to 5, 6-dihydrouracil then dihydropyrimidinase hydrolyzes 5, 6-dihydrouracil to N-carbamyl-b-alanine. Finally, beta-ureidopropionase catalyzes the conversion of N-carbamyl-b-alanine to beta-alanine. Patients with dihydropyrimidinase deficiency (DPYS; also called 5, 6-dihydropyrimidine amidohydrolase, or DHP; EC 3.5.2.2) exhibit highly increased concentrations of 5, 6-dihydrouracil in urine. The direct measurement of the activity of DHP in patients had been hampered by the fact that the enzyme is expressed almost exclusively in liver tissue. Various neurological abnormalities have been described in this group of patients. (OMIM 222748)

Biological Function

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Catabolism

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Diseases / Conditions Related to Nutrition

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  • Dihydropyrimidine dehydrogenase (DPD) deficiency

Other (Monogenic) Disorders

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Nutritional Information

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Drivers for biological variation

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Vulnerable groups

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Other resources

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Links

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