Succinylacetone

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Succinylacetone
2D structure for Succinylacetone
Chemical Name 4,6-dioxoheptanoic acid
Chemical Formula C7H10O4
CAS Number 51568-18-4
Chemical Information HMDB00635
Biochemical Taxonomy

  • Keto-Acids

Functional Taxonomy Not Available
Nutritional Taxonomy Not Available
Metabolic Pathways Not Available
Biofluid Location

  • Blood

Tissue Location Not Available
Normal Biofluid Concentrations

  • Blood: 0.013 (0.003-0.021) umol/L

Normal Tissue Concentrations Not Available
Diseases / Conditions Related to Nutrition

  • Patients with tyrosinemia type I

Other (Monogenic Disorders) Not Available
Abnormal Biofluid Concentrations

  • Blood (Patients with tyrosinemia type I): 0.033 (0.021-0.055) umol/L

Abnormal Tissue Concentrations Not Available
Physiological Processes Not Available
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Contents

Introduction

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Succinylacetone is a tyrosine metabolite (PMID 16448836). It is a specific marker for Tyrosinemia type I. Type I tyrosinemia is an inherited metabolism disorder due to a shortage of the enzyme fumarylacetoacetate hydrolase that is needed to break down tyrosine. (Wikipedia)

Biological Function

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Catabolism

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Diseases / Conditions Related to Nutrition

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  • Patients with tyrosinemia type I

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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