3-Hydroxyglutaric acid

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3-Hydroxyglutaric acid
2D structure for 3-Hydroxyglutaric acid
Chemical Name 3-hydroxypentanedioic acid
Chemical Formula C5H8O5
CAS Number 638-18-6
Chemical Information HMDB00428
Biochemical Taxonomy

  • Dicarboxylic Acids

Functional Taxonomy Not Available
Nutritional Taxonomy Not Available
Metabolic Pathways

  • Lysine Degradation

Biofluid Location

  • Blood

Tissue Location

  • Neurons

Normal Biofluid Concentrations

  • Blood: 0.15 +/- 0.08 umol/L

Normal Tissue Concentrations Not Available
Diseases / Conditions Related to Nutrition Not Available
Other (Monogenic Disorders) Not Available
Abnormal Biofluid Concentrations Not Available
Abnormal Tissue Concentrations Not Available
Physiological Processes Not Available
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Contents

Introduction

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3-Hydroxyglutaric acid is a key metabolite in glutaryl co-enzyme A dehydrogenase deficiency, and is considered to be a potential neurotoxin. The urine level of 3-Hydroxyglutaric acid is elevated in Glutaric Aciduria Type I (glutaryl-CoA dehydrogenase deficiency) patients. (PMID 16573641)

Biological Function

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Catabolism

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Diseases / Conditions Related to Nutrition

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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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