Glycine

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Glycine
2D structure for Glycine
Chemical Name	2 aminoacetic acid
Chemical Formula	C₄₇H₈₄O₂
CAS Number	56-40-6
Chemical Information	HMDB00123
Biochemical Taxonomy	Amino Acids Nitrogenous Compounds
Functional Taxonomy	Not available
Nutritional Taxonomy	Non-Essential Amino Acids
Metabolic Pathways	One Carbon Pool by Folate Folate Biosynthesis
Biofluid Location	Urine Bile Cerebrospinal Fluid (CSF) Saliva Blood
Tissue Location	Bladder Brain Epidermis Fibroblasts Intestine Kidney Myelin Neurons Pancreas Placenta Platelet Prostate Spleen Stratum Corneum Thyroid Gland
Normal Biofluid Concentrations	Urine: 513.54 +/- 360.61 umol/mmol_creatinine Cerebrospinal Fluid (CSF): 4.7 +/- 1.5 uM Blood: 280.0 (140.0-420.0) uM Saliva: >10 umol/L Blood: 230.0 (178.0-282.0) umol/L Cerebrospinal Fluid (CSF): 8.30 (5.88-10.7) umol/L Bile: >10 umol/L Urine: 151.0 (480.0-233.0) umol/mmol_creatinine Cerebrospinal Fluid (CSF): 9.0 (3.0-19.0) umol/L
Normal Tissue Concentrations	Not available
Diseases / Conditions Related to Nutrition	Lung Cancer Non-ketotic hyperglycinemia leukemia leukemia with CNS disease Juvenile myoclonic epilepsy (JME) Refractory localization-related epilepsy (RLE) Diabetes
Other (Monogenic Disorders)	Glycine encephalopathy; GCE; Hyperglycinemia, nonketotic; NKH; Hyperglycinemia, transient neonatal, included; TNH, included OMIM: 605899
Abnormal Biofluid Concentrations	Blood (Non-ketotic hyperglycinemia): 1090.0 (780.0-1400.0) uM Blood (Diabetes): 170 umol/L Blood (Refractory localization-related epilepsy (RLE)): 210.0 (203.0-217.0) umol/L Blood (Juvenile myoclonic epilepsy (JME)): 354.0 (309.0-399.0) umol/L Urine (Lung Cancer): 84.0 +/- 67.0 umol/mmol_creatinine Cerebrospinal Fluid (CSF) (leukemia): 9.7 +/- 4.9 umol/L Cerebrospinal Fluid (CSF) (leukemia with CNS disease): 13.4 +/- 6.4 umol/L
Abnormal Tissue Concentrations	Not available
Physiological Processes	Not available

Authors:
Affiliations:

1 Introduction
2 Biological Function
3 Catabolism
4 Diseases / Conditions Related to Nutrition
- 4.1 Associated decreased protein/metabolite profile
- 4.2 Associated increased protein/metabolite profile
5 Other (Monogenic) Disorders
6 Nutritional Information
7 Drivers for biological variation
8 Vulnerable groups
9 Other resources
10 Links

Introduction

guidelines
Glycine is a simple, nonessential amino acid, although experimental animals show reduced growth on low-glycine diets. The average adult ingests 3 to 5 grams of glycine daily. Glycine is involved in the body's production of DNA, phospholipids and collagen, and in release of energy. Glycine levels are effectively measured in plasma in both normal patients and those with inborn errors of glycine metabolism. (http://www.dcnutrition.com/AminoAcids/) Nonketotic hyperglycinaemia (OMIM 606899) is an autosomal recessive condition caused by deficient enzyme activity of the glycine cleavage enzyme system (EC 2.1.1.10). The glycine cleavage enzyme system comprises four proteins: P-, T-, H- and L-proteins (EC 1.4.4.2, EC 2.1.2.10 and EC 1.8.1.4 for P-, T- and L-proteins). Mutations have been described in the GLDC (OMIM 238300), AMT (OMIM 238310), and GCSH (OMIM 238330) genes encoding the P-, T-, and H-proteins respectively. The glycine cleavage system catalyses the oxidative conversion of glycine into carbon dioxide and ammonia, with the remaining one-carbon unit transferred to folate as methylenetetrahydrofolate. It is the main catabolic pathway for glycine and it also contributes to one-carbon metabolism. Patients with a deficiency of this enzyme system have increased glycine in plasma, urine and cerebrospinal fluid (CSF) with an increased CSF: plasma glycine ratio. (PMID 16151895)