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Citrulline
2D structure for Citrulline
Chemical Name	(2S)-2-amino-5-(carbamoylamino)pentanoic acid
Chemical Formula	C6H13N3O3
CAS Number	372-75-8
Chemical Information	HMDB00904
Biochemical Taxonomy	Amino Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Alanine and Aspartate Metabolism Glycine, Serine and Threonine Metabolism
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Urine
Tissue Location	Epidermis Fibroblasts Intestine Kidney Liver Myelin Nerves Neurons Placenta Platelet Bladder
Normal Biofluid Concentrations	Blood: 35.0 +/- 10.0 uM Blood: 36.0 +/- 8.0 uM Blood: 37.0 +/- 9.0 uM Blood: 38.0 (30.0-46.0) uM Cerebrospinal Fluid (CSF): 1.5 +/- 0.5 uM Cerebrospinal Fluid (CSF): 2.62 (1.35-3.89) uM Cerebrospinal Fluid (CSF): 2.8 +/- 1.8 uM Urine: 0.50 +/- 0.54 umol/mmol creatinine Urine: 0.99 +/- 0.69 umol/mmol creatinine Urine: 1.08 (0.065-2.1) umol/mmol creatinine Urine: 1.1 +/- 0.84 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Acute seizures Juvenile myoclonic epilepsy (JME) Normal Permanent Intestinal Failure Probable Alzheimer's Disease Refractory localization-related epilepsy (RLE)
Other (Monogenic Disorders)	Argininemia OMIM: 207800 Argininosuccinic aciduria OMIM: 207900 Carbamoyl phosphate synthetase i deficiency, hyperammonemia due to OMIM: 237300 Citrullinemia, classic OMIM: 215700 Citrullinemia, type II, adult-onset OMIM: 603471 Citrullinemia, type II, neonatal-onset OMIM: 605814 Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome OMIM: 238970 Lysinuric protein intolerance OMIM: 222700 Ornithine transcarbamylase deficiency, hyperammonemia due to OMIM: 311250
Abnormal Biofluid Concentrations	Blood (Acute seizures): 25.4 (23.4-27.4) uM Blood (Juvenile myoclonic epilepsy (JME)): 26.9 (25.2-28.6) uM Blood (Permanent Intestinal Failure): 13.0 (6.0-20.0) uM Blood (Probable Alzheimer's Disease): 94.97 +/- 11.18 uM Blood (Refractory localization-related epilepsy (RLE)): 26.8 (25.4-28.2) uM Cerebrospinal Fluid (CSF) (Normal): 2.8 +/- 1.0 uM
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Contents 1 Introduction 2 Biological Function 3 Catabolism 4 Diseases / Conditions Related to Nutrition 5 Other (Monogenic) Disorders 6 Nutritional Information 7 Drivers for biological variation 8 Vulnerable groups 9 Other resources 10 Links

Introduction

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Citrulline is an amino acid made from ornithine and carbamoyl phosphate in one of the central reactions in the urea cycle. It is also produced from arginine as a by-product of the reaction catalyzed by NOS family (NOS; EC 1.14.13.39). In this reaction arginine is first oxidized into N-hydroxyl-arginine, which is then further oxidized to citrulline concomitant with the release of nitric oxide.; EC 1.14.13.39). Citrulline's name is derived from citrullus, the Latin word for watermelon, from which it was first isolated.

Biological Function

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Catabolism

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Diseases / Conditions Related to Nutrition

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• Acute seizures
• Juvenile myoclonic epilepsy (JME)
• Normal
• Permanent Intestinal Failure
• Probable Alzheimer's Disease
• Refractory localization-related epilepsy (RLE)

Other (Monogenic) Disorders

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• Argininemia OMIM: 207800
• Argininosuccinic aciduria OMIM: 207900
• Carbamoyl phosphate synthetase i deficiency, hyperammonemia due to OMIM: 237300
• Citrullinemia, classic OMIM: 215700
• Citrullinemia, type II, adult-onset OMIM: 603471
• Citrullinemia, type II, neonatal-onset OMIM: 605814
• Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome OMIM: 238970
• Lysinuric protein intolerance OMIM: 222700
• Ornithine transcarbamylase deficiency, hyperammonemia due to OMIM: 311250

Nutritional Information

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Drivers for biological variation

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The levels of the two non-proteinogenic amino acids that participate in the hepatic urea synthesis are associated with glucose homeostasis, shown by Shaham et al (PubMed). These two amino acids(in plasma) decreased by 35 and 29% respectively in response to an oral glucose challenge test. The decreases in citrulline and ornithine may be associated with the reduction in gluconeogenesis and urea synthesis following glucose ingestion as gluconeogenesis from amino acids (primarily from alanine)supports 25-40% of the non-glygogen-derived hepatic glucose output after an overnight fast.

Vulnerable groups

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Other resources

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Links

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