Ketoleucine

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Ketoleucine
2D structure for Ketoleucine
Chemical Name	4-methyl-2-oxopentanoic acid
Chemical Formula	C₆H₁₀O₃
CAS Number	816-66-0
Chemical Information	HMDB00695
Biochemical Taxonomy	Keto-Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Pantothenate and CoA Biosynthesis Valine, Leucine and Isoleucine Biosynthesis Valine, Leucine and Isoleucine Degradation
Biofluid Location	Blood Cerebrospinal Fluid (CSF)
Tissue Location	Neurons Muscle
Normal Biofluid Concentrations	Blood: 28.0 (0.0-58.0) uM Blood: 33.5 +/- 8.2 uM Cerebrospinal Fluid (CSF): 5.0 +/- 4.4 uM
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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Introduction

guidelines
Ketoleucine is a metabolite that accumulates in Maple Syrup Urine Disease (MSUD) and shown to compromise brain energy metabolism by blocking the respiratory chain; this is of relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients. (PMID 14636955)