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Human Metabolome Database Version 2.5

 

Showing metabocard for Glycocholic acid (HMDB00138)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2010-07-13 12:13:53
Accession Number HMDB00138
Secondary Accession Numbers Not Available
Common Name Glycocholic acid
Description Glycocholic acid is an acyl glycine and a bile acid-glycine conjugate. It is a secondary bile acid produced by the action of enzymes existing in the microbial flora of the colonic environment. In hepatocytes, both primary and secondary bile acids undergo amino acid conjugation at the C-24 carboxylic acid on the side chain, and almost all bile acids in the bile duct therefore exist in a glycine conjugated form (PMID:16949895). More specifically, glycocholic acid or cholylglycine, is a crystalline bile acid involved in the emulsification of fats. It occurs as a sodium salt in the bile of mammals. Its anion is called glycocholate. As the glycine conjugate of cholic acid, this compound acts as a detergent to solubilize fats for absorption and is itself absorbed. (PubChem). Bile acids are steroid acids found predominantly in bile of mammals. The distinction between different bile acids is minute, depends only on presence or absence of hydroxyl groups on positions 3, 7, and 12. Bile acids are physiological detergents that facilitate excretion, absorption, and transport of fats and sterols in the intestine and liver. Bile acids are also steroidal amphipathic molecules derived from the catabolism of cholesterol. They modulate bile flow and lipid secretion, are essential for the absorption of dietary fats and vitamins, and have been implicated in the regulation of all the key enzymes involved in cholesterol homeostasis. Bile acids recirculate through the liver, bile ducts, small intestine and portal vein to form an enterohepatic circuit. They exist as anions at physiological pH and, consequently, require a carrier for transport across the membranes of the enterohepatic tissues. The unique detergent properties of bile acids are essential for the digestion and intestinal absorption of hydrophobic nutrients. Bile acids have potent toxic properties (e.g., membrane disruption) and there are a plethora of mechanisms to limit their accumulation in blood and tissues. (PMID: 11316487, 16037564, 12576301, 11907135)
Synonyms
  1. 3a,7a,12a-Trihydroxy-5b-cholan-24-oylglycine
  2. 3a,7a,12a-Trihydroxy-5b-cholanic acid-24-glycine
  3. 3a,7a,12a-Trihydroxy-N-(carboxymethyl)-5b-cholan-24-amide
  4. Cholylglycine
  5. Glycine cholate
  6. Glycocholate
  7. Glycocholic acid
  8. Glycoreductodehydrocholic acid
  9. Glycylcholate
  10. Glycylcholic acid
  11. N-(carboxymethyl)-3a,7a,12a-trihydroxy-5b-Cholan-24-amide
  12. N-Choloylglycine
  13. N-choloyl-Glycine
Chemical IUPAC Name 2-[4-(3,7,12-trihydroxy-10,13-dimethyl-2,3,4,5,6,7,8,9,11,12,14,15,16,17-tetradecahydro-1H-cyclopenta[a]phenanthren-17-yl)pentanoylamino]acetic acid
Chemical Formula C26H43NO6
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Bile Acids
  • Acyl Glycines
Sub Class
  • Bile-acid glycine conjugates
Family
  • Mammalian Metabolite
Species
  • secondary alcohol
  • carboxylic acid
  • secondary carboxylic acid amide
Biofunction
—
Application
—
Source
  • Endogenous
Average Molecular Weight 465.623
Monoisotopic Molecular Weight 465.309052
Isomeric SMILES C[C@H](CCC(=O)NCC(O)=O)[C@H]1CC[C@H]2[C@@H]3[C@H](O)CC4C[C@H](O)CC[C@]4(C)[C@H]3C[C@H](O)[C@]12C
Canonical SMILES CC(CCC(=O)NCC(O)=O)C1CCC2C3C(O)CC4CC(O)CCC4(C)C3CC(O)C12C
KEGG Compound ID C01921 Link Image
BioCyc ID GLYCOCHOLIC_ACID Link Image
BiGG ID 38691 Link Image
Wikipedia Link Glycocholic acid Link Image
NuGOwiki Link HMDB00138 Link Image
Metagene Link HMDB00138 Link Image
METLIN ID 5169 Link Image
PubChem Compound 439604 Link Image
PubChem Substance 5027 Link Image
ChEBI ID 29746 Link Image
CAS Registry Number 475-31-0
InChI Identifier InChI=1/C26H43NO6/c1-14(4-7-22(31)27-13-23(32)33)17-5-6-18-24-19(12-21(30)26(17,18)3)25(2)9-8-16(28)10-15(25)11-20(24)29/h14-21,24,28-30H,4-13H2,1-3H3,(H,27,31)(H,32,33)/t14-,15?,16-,17-,18+,19+,20-,21+,24+,25+,26-/m1/s1
Synthesis Reference Cortese, Frank; Bauman, Louis. A synthesis of conjugated bile acids. I. Glycocholic acid. Journal of the American Chemical Society (1935), 57 1393-5.
Melting Point (Experimental) 170 oC
Experimental Water Solubility 0.0033 mg/mL [YALKOWSKY,SH & DANNENFELSER,RM (1992)] Source: PhysProp
Predicted Water Solubility 0.0248 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity 1.65 [RODA,A ET AL. (1990)] Source: PhysProp
Predicted LogP/Hydrophobicity 1.70 [Predicted by ALOGPS]; 1.1 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID 1EIO Link Image
Experimental PDB File Show
Experimental PDB Structure
Experimental 1H NMR Spectrum Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
Show Experimental Conditions Link Image
Medium Energy
Download File
Show Experimental Conditions Link Image
High Energy
Download File
Show Experimental Conditions Link Image
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
  • Extracellular
  • peroxisome
Biofluid Location
  • Blood
  • Urine
Tissue Location
Tissue References
Fibroblasts —
Liver —
Concentrations (Normal)
Biofluid Blood
Value 0.88 +/- 0.26 uM
Age Children:1-13 yrs old
Sex N/A
Patient information Normal
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 0.06 +/- 0.04 uM
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Spiller RC, Frost PF, Stewart JS, Bloom SR, Silk DB: Delayed postprandial plasma bile acid response in coeliac patients with slow mouth-caecum transit. Clin Sci (Lond). 1987 Feb;72(2):217-23. [PubMed Link Image]
Biofluid Urine
Value 0.42 +/- 0.36 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
  • Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed Link Image]
Concentrations (Abnormal)
Biofluid Blood
Value 43.6 +/- 11.8 uM
Age Adult:>18 yrs old
Sex Both
Condition Hepatobiliary diseases
Comments Not Available
References
  • Rifai K, Ockenga J, Manns MP, Bischoff SC: Repeated administration of a vitamin preparation containing glycocholic acid in patients with hepatobiliary disease. Aliment Pharmacol Ther. 2006 May 1;23(9):1337-45. [PubMed Link Image]
Biofluid Blood
Value 0.7 +/- 0.13 uM
Age Adult:>18 yrs old
Sex Both
Condition Celiac disease
Comments Not Available
References
  • Spiller RC, Frost PF, Stewart JS, Bloom SR, Silk DB: Delayed postprandial plasma bile acid response in coeliac patients with slow mouth-caecum transit. Clin Sci (Lond). 1987 Feb;72(2):217-23. [PubMed Link Image]
Biofluid Blood
Value 44.0 +/- 29.1 uM
Age Children:1-13 yrs old
Sex N/A
Condition Biliary atresia
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 8.4 +/- 8.7 uM
Age Children:1-13 yrs old
Sex N/A
Condition Choledochal cysts
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 91.8 +/- 54.9 uM
Age Adult:>18 yrs old
Sex N/A
Condition Intrahepatic biliary hypoplasia
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 2.8 +/- 3.1 uM
Age Children:1-13 yrs old
Sex N/A
Condition Neonatal hepatitis
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 25.9 +/- 24.0 uM
Age Children:1-13 yrs old
Sex N/A
Condition Alpha-1-antitrypsin deficiency
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 9.8 +/- 14.7 uM
Age Children:1-13 yrs old
Sex N/A
Condition Cystic fibrosis
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 2.9 +/- 2.4 uM
Age Children:1-13 yrs old
Sex N/A
Condition Galactosemia type 1
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 14.5 +/- 16.1 uM
Age Children:1-13 yrs old
Sex N/A
Condition Chronic active hepatitis
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 8.6 +/- 7.9 uM
Age Children:1-13 yrs old
Sex N/A
Condition Glycogen storage disease
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 1.5 +/- 1.2 uM
Age Children:1-13 yrs old
Sex N/A
Condition Acute liver failure
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 10.5 +/- 13.1 uM
Age Children:1-13 yrs old
Sex N/A
Condition Portal vein obstruction
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 5.1 +/- 6.9 uM
Age Children:1-13 yrs old
Sex N/A
Condition Wilson's disease
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Blood
Value 11.3 +/- 26.1 uM
Age Children:1-13 yrs old
Sex N/A
Comments Not Available
References
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biofluid Urine
Value 1.55 +/- 1.22 umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Abnormal
Comments Not Available
References
  • Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed Link Image]
Associated Disorders
Condition References
Acute liver failure
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Alpha-1-antitrypsin deficiency
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Biliary atresia
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Celiac disease
  • Spiller RC, Frost PF, Stewart JS, Bloom SR, Silk DB: Delayed postprandial plasma bile acid response in coeliac patients with slow mouth-caecum transit. Clin Sci (Lond). 1987 Feb;72(2):217-23. [PubMed Link Image]
Choledochal cysts
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Chronic active hepatitis
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Cystic fibrosis
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Galactosemia type 1
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Glycogen storage disease
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Hepatobiliary diseases
  • Rifai K, Ockenga J, Manns MP, Bischoff SC: Repeated administration of a vitamin preparation containing glycocholic acid in patients with hepatobiliary disease. Aliment Pharmacol Ther. 2006 May 1;23(9):1337-45. [PubMed Link Image]
Intrahepatic biliary hypoplasia
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Neonatal hepatitis
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Portal vein obstruction
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
Wilson's disease
  • Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
OMIM ID
Pathways
Name SMPDB Link KEGG Link
Bile Acid Biosynthesis SMP00035 Link Image map00120 Link Image
General References
  1. Spiller RC, Frost PF, Stewart JS, Bloom SR, Silk DB: Delayed postprandial plasma bile acid response in coeliac patients with slow mouth-caecum transit. Clin Sci (Lond). 1987 Feb;72(2):217-23. [PubMed Link Image]
  2. Matsui A, Psacharopoulos HT, Mowat AP, Portmann B, Murphy GM: Radioimmunoassay of serum glycocholic acid, standard laboratory tests of liver function and liver biopsy findings: comparative study of children with liver disease. J Clin Pathol. 1982 Sep;35(9):1011-7. [PubMed Link Image]
  3. Marigold JH, Gilmore IT, Thompson RP: Effects of a meal on plasma clearance of [14C]glycocholic acid and indocyanine green in man. Clin Sci (Lond). 1981 Sep;61(3):325-30. [PubMed Link Image]
  4. Xia S, Chen Z, Li L: [Relationship of vascular endothelial growth factor with bile acid in intrahepatic cholestasis of pregnancy] Zhonghua Fu Chan Ke Za Zhi. 2002 Nov;37(11):669-71. [PubMed Link Image]
  5. Rius M, Nies AT, Hummel-Eisenbeiss J, Jedlitschky G, Keppler D: Cotransport of reduced glutathione with bile salts by MRP4 (ABCC4) localized to the basolateral hepatocyte membrane. Hepatology. 2003 Aug;38(2):374-84. [PubMed Link Image]
  6. Li GY, Wang T, Huggins EM Jr, Shams NK, Davis JF, Calkins JH, Hornung CA, Altekruse JM, Sigel MM: Cholylglycine measured in serum by RIA and interleukin-1 beta determined by ELISA in differentiating viral hepatitis from chemical liver injury. J Occup Med. 1992 Sep;34(9):930-3. [PubMed Link Image]
  7. de Franchis R, Vecchi M, Primignani M, Bonato C, Parravicini A, Cambieri R, Ciaci D, Annoni G: Diagnostic value of serum cholylglycine radioimmunoassay in chronic asymptomatic HBsAg carriers. Ric Clin Lab. 1983 Jul-Sep;13(3):301-5. [PubMed Link Image]
  8. Xuan B, McClellan DA, Moore R, Chiou GC: Alternative delivery of insulin via eye drops. Diabetes Technol Ther. 2005 Oct;7(5):695-8. [PubMed Link Image]
  9. Liss GM, Greenberg RA, Tamburro CH: Use of serum bile acids in the identification of vinyl chloride hepatotoxicity. Am J Med. 1985 Jan;78(1):68-76. [PubMed Link Image]
  10. Rifai K, Ockenga J, Manns MP, Bischoff SC: Repeated administration of a vitamin preparation containing glycocholic acid in patients with hepatobiliary disease. Aliment Pharmacol Ther. 2006 May 1;23(9):1337-45. [PubMed Link Image]
  11. Klapdor R: On the kinetics of glycocholate uptake and excretion by the normal and diseased liver in man. Hepatogastroenterology. 1981 Aug;28(4):189-91. [PubMed Link Image]
  12. Friman S, Radberg G, Bosaeus I, Svanvik J: Hepatobiliary compensation for the loss of gallbladder function after cholecystectomy. An experimental study in the cat. Scand J Gastroenterol. 1990 Mar;25(3):307-14. [PubMed Link Image]
  13. Bocharova LV, Tsodikov GV, Chernyshova NN, Kataev SS, Burkov SG: [Clinical value of determining serum levels of glycocholic acid in alcoholic lesions of the liver] Klin Med (Mosk). 1992 Jul-Aug;70(7-8):41-4. [PubMed Link Image]
  14. Murphy JL, Badaloo AV, Chambers B, Forrester TE, Wootton SA, Jackson AA: Maldigestion and malabsorption of dietary lipid during severe childhood malnutrition. Arch Dis Child. 2002 Dec;87(6):522-5. [PubMed Link Image]
  15. Bremmelgaard A, Ranek L, Bahnsen M, Andreasen PB, Christensen E: Cholic acid conjugation test and quantitative liver function in acute liver failure. Scand J Gastroenterol. 1983 Sep;18(6):797-802. [PubMed Link Image]
  16. Hepner GW, Demers LM: Dynamics of the enterohepatic circulation of the glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and sulfolithocholic acid in man. Gastroenterology. 1977 Mar;72(3):499-501. [PubMed Link Image]
  17. Kasatkin IuN, Vidiukov VI, Mironov SP, Chernyshova NN, Bocharova LV, Kataev SS: [Diagnosis of liver cirrhosis based on scintigraphic data and the level of cholylglycine in the blood serum] Med Radiol (Mosk). 1989 Sep;34(9):8-12. [PubMed Link Image]
  18. Collazos J, Mendarte U, De Miguel J: Clinical value of the determination of fasting glycocholic acid serum levels in patients with liver diseases. A comparison with standard liver tests. Gastroenterol Clin Biol. 1993;17(2):79-82. [PubMed Link Image]
  19. Nittono H, Obinata K, Nakatsu N, Watanabe T, Niijima S, Sasaki H, Arisaka O, Kato H, Yabuta K, Miyano T: Sulfated and nonsulfated bile acids in urine of patients with biliary atresia: analysis of bile acids by high-performance liquid chromatography. J Pediatr Gastroenterol Nutr. 1986 Jan;5(1):23-9. [PubMed Link Image]
  20. Wikipedia Link Image
Metabolic Enzymes
  1. Bile acid CoA:amino acid N-acyltransferase
  2. Glycine N-acyltransferase
  3. Glycine N-acyltransferase-like protein 1
  4. Glycine N-acyltransferase-like protein 2
  5. Glycine N-acyltransferase-like protein 3
Enzyme 1 [top]
Enzyme 1 ID 5821
Enzyme 1 Name Bile acid CoA:amino acid N-acyltransferase
Enzyme 1 Synonyms
  1. BAT
  2. BACAT
  3. Glycine N-choloyltransferase
  4. Long-chain fatty-acyl-CoA hydrolase
Enzyme 1 Gene Name BAAT
Enzyme 1 Protein Sequence >Bile acid CoA:amino acid N-acyltransferase 
MIQLTATPVSALVDEPVHIRATGLIPFQMVSFQASLEDENGDMFYSQAHYRANEFGEVDL
NHASSLGGDYMGVHPMGLFWSLKPEKLLTRLLKRDVMNRPFQVQVKLYDLELIVNNKVAS
APKASLTLERWYVAPGVTRIKVREGRLRGALFLPPGEGLFPGVIDLFGGLGGLLEFRASL
LASRGFASLALAYHNYEDLPRKPEVTDLEYFEEAANFLLRHPKVFGSGVGVVSVCQGVQI
GLSMAIYLKQVTATVLINGTNFPFGIPQVYHGQIHQPLPHSAQLISTNALGLLELYRTFE
TTQVGASQYLFPIEEAQGQFLFIVGEGDKTINSKAHAEQAIGQLKRHGKNNWTLLSYPGA
GHLIEPPYSPLCCASTTHDLRLHWGGEVIPHAAAQEHAWKEIQRFLRKHLIPDVTSQL
Enzyme 1 Number of Residues 418
Enzyme 1 Molecular Weight 46300
Enzyme 1 Theoretical pI 7.00
Enzyme 1 GO Classification
Function
  • CoA hydrolase activity
  • catalytic activity
  • hydrolase activity
  • hydrolase activity, acting on ester bonds
  • palmitoyl-CoA hydrolase activity
  • thiolester hydrolase activity
Process
  • lipid metabolism
  • metabolism
  • physiological process
  • primary metabolism
Component
—
Enzyme 1 General Function Not Available
Enzyme 1 Specific Function Involved in bile acid metabolism. In liver hepatocytes catalyzes the second step in the conjugation of C24 bile acids (choloneates) to glycine and taurine before excretion into bile canaliculi. The major components of bile are cholic acid and chenodeoxycholic acid. In a first step the bile acids are converted to an acyl-CoA thioester, either in peroxisomes (primary bile acids deriving from the cholesterol pathway), or cytoplasmic at the endoplasmic reticulum (secondary bile acids). May catalyze the conjugation of primary or secondary bile acids, or both. The conjugation increases the detergent properties of bile acids in the intestine, which facilitates lipid and fat-soluble vitamin absorption. In turn, bile acids are deconjugated by bacteria in the intestine and are recycled back to the liver for reconjugation (secondary bile acids). May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids. In vitro, catalyzes the hydrolysis of long- and very long-chain saturated acyl-CoAs to the free fatty acid and coenzyme A (CoASH), and conjugates glycine to these acyl-CoAs
Enzyme 1 Pathways
Enzyme 1 Reactions
  • palmitoyl-CoA + H2O = CoA + palmitate
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 532505 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q14032 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name BAAT_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence >1257 bp 
ATGATCCAGTTGACAGCTACCCCTGTGAGTGCACTTGTTGATGAGCCAGTGCATATCCGA
GCTACAGGCCTGATTCCCTTTCAGATGGTGAGTTTTCAGGCATCACTGGAAGATGAAAAC
GGAGACATGTTTTATTCTCAAGCCCACTATAGGGCCAATGAATTCGGTGAGGTGGACCTG
AATCATGCTTCTTCACTTGGAGGGGATTATATGGGAGTCCACCCCATGGGTCTCTTCTGG
TCTCTGAAACCTGAAAAGCTATTAACAAGACTGTTGAAAAGAGATGTGATGAATAGGCCT
TTCCAGGTCCAAGTAAAACTTTATGACTTAGAGTTAATAGTGAACAATAAAGTTGCCAGT
GCTCCAAAGGCCAGCCTGACTTTGGAGAGGTGGTATGTGGCACCTGGTGTCACACGAATT
AAGGTTCGAGAAGGCCGCCTTCGAGGAGCTCTCTTTCTCCCTCCAGGAGAGGGTCTCTTC
CCAGGGGTAATTGATTTGTTTGGTGGTTTGGGTGGGCTGCTTGAATTTCGGGCCAGCCTC
CTAGCCAGTCGTGGCTTCGCCTCCTTGGCCTTGGCTTACCATAACTATGAAGACCTGCCC
CGCAAACCAGAAGTAACAGATTTGGAATATTTTGAGGAGGCTGCCAACTTTCTCCTGAGA
CATCCAAAGGTCTTTGGCTCAGGCGTTGGGGTAGTCTCTGTATGTCAAGGAGTACAGATT
GGACTATCTATGGCTATTTACCTAAAGCAAGTCACAGCCACGGTACTTATTAATGGGACC
AACTTTCCTTTTGGCATTCCACAGGTATATCATGGTCAGATCCATCAGCCCCTTCCCCAT
TCTGCACAATTAATATCCACCAATGCCTTGGGGTTACTAGAGCTCTATCGCACTTTTGAG
ACAACTCAAGTTGGGGCCAGTCAATATTTGTTTCCTATTGAAGAGGCCCAGGGGCAATTC
CTCTTCATTGTAGGAGAAGGTGATAAGACTATCAACAGCAAAGCACACGCTGAACAAGCC
ATAGGACAGCTGAAGAGACATGGGAAGAACAACTGGACCCTGCTATCTTACCCTGGGGCA
GGCCACCTGATAGAACCTCCCTATTCTCCTCTGTGCTGTGCCTCAACGACCCACGATTTG
AGGTTACACTGGGGAGGAGAGGTGATCCCACACGCAGCTGCACAGGAACATGCTTGGAAG
GAGATCCAGAGATTTCTCAGGAAGCACCTCATTCCAGATGTGACCAGTCAACTCTAA
Enzyme 1 GenBank Gene ID L34081 Link Image
Enzyme 1 GeneCard ID BAAT Link Image
Enzyme 1 GenAtlas ID BAAT Link Image
Enzyme 1 HGNC ID HGNC:932 Link Image
Enzyme 1 Chromosome Location 9
Enzyme 1 Locus 9q22.3
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References
  1. Falany CN, Johnson MR, Barnes S, Diasio RB: Glycine and taurine conjugation of bile acids by a single enzyme. Molecular cloning and expression of human liver bile acid CoA:amino acid N-acyltransferase. J Biol Chem. 1994 Jul 29;269(30):19375-9. [PubMed Link Image]
  2. Carlton VE, Harris BZ, Puffenberger EG, Batta AK, Knisely AS, Robinson DL, Strauss KA, Shneider BL, Lim WA, Salen G, Morton DH, Bull LN: Complex inheritance of familial hypercholanemia with associated mutations in TJP2 and BAAT. Nat Genet. 2003 May;34(1):91-6. [PubMed Link Image]
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 12971
Enzyme 2 Name Glycine N-acyltransferase
Enzyme 2 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase
  2. AAc
  3. Aralkyl acyl-CoA N-acyltransferase
  4. Aralkyl acyl-CoA:amino acid N-acyltransferase
  5. HRP-1(CLP)
Enzyme 2 Gene Name GLYAT
Enzyme 2 Protein Sequence >Glycine N-acyltransferase 
MMLPLQGAQMLQMLEKSLRKSLPASLKVYGTVFHINHGNPFNLKAVVDKWPDFNTVVVCP
QEQDMTDDLDHYTNTYQIYSKDPQNCQEFLGSPELINWKQHLQIQSSQPSLNEAIQNLAA
IKSFKVKQTQRILYMAAETAKELTPFLLKSKILSPSGGKPKAINQEMFKLSSMDVTHAHL
VNKFWHFGGNERSQRFIERCIQTFPTCCLLGPEGTPVCWDLMDQTGEMRMAGTLPEYRLH
GLVTYVIYSHAQKLGKLGFPVYSHVDYSNEAMQKMSYTLQHVPIPRSWNQWNCVPL
Enzyme 2 Number of Residues 296
Enzyme 2 Molecular Weight 33898
Enzyme 2 Theoretical pI 8.28
Enzyme 2 GO Classification Not Available
Enzyme 2 General Function Not Available
Enzyme 2 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 2554941 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q6IB77 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name GLYAT_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID AF023466 Link Image
Enzyme 2 GeneCard ID Q6IB77 Link Image
Enzyme 2 GenAtlas ID GLYAT Link Image
Enzyme 2 HGNC ID HGNC:13734 Link Image
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available
Enzyme 3 [top]
Enzyme 3 ID 12972
Enzyme 3 Name Glycine N-acyltransferase-like protein 1
Enzyme 3 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase-like protein 1
Enzyme 3 Gene Name GLYATL1
Enzyme 3 Protein Sequence >Glycine N-acyltransferase-like protein 1 
MILLNNSHKLLALYKSLARSIPESLKVYGSVYHINHGNPFNMEVLVDSWPEYQMVIIRPQ
KQEMTDDMDSYTNVYRMFSKEPQKSEEVLKNCEIVNWKQRLQIQGLQESLGEGIRVATFS
KSVKVEHSRALLLVTEDILKLNASSKSKLGSWAETGHPDDEFESETPNFKYAQLDVSYSG
LVNDNWKRGKNERSLHYIKRCIEDLPAACMLGPEGVPVSWVTMDPSCEVGMAYSMEKYRR
TGNMARVMVRYMKYLRQKNIPFYISVLEENEDSRRFVGQFGFFEASCEWHQWTCYPQNLV
PF
Enzyme 3 Number of Residues 302
Enzyme 3 Molecular Weight 35102
Enzyme 3 Theoretical pI 6.86
Enzyme 3 GO Classification Not Available
Enzyme 3 General Function Not Available
Enzyme 3 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 3 Pathways Not Available
Enzyme 3 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 3 Pfam Domain Function
Enzyme 3 Signals
  • None
Enzyme 3 Transmembrane Regions
  • None
Enzyme 3 Essentiality Not Available
Enzyme 3 GenBank ID Protein 71384826 Link Image
Enzyme 3 UniProtKB/Swiss-Prot ID Q969I3 Link Image
Enzyme 3 UniProtKB/Swiss-Prot Entry Name GLYL1_HUMAN Link Image
Enzyme 3 PDB ID Not Available
Enzyme 3 Cellular Location Not Available
Enzyme 3 Gene Sequence Not Available
Enzyme 3 GenBank Gene ID DQ084381 Link Image
Enzyme 3 GeneCard ID Q969I3 Link Image
Enzyme 3 GenAtlas ID GLYATL1 Link Image
Enzyme 3 HGNC ID HGNC:30519 Link Image
Enzyme 3 Chromosome Location Not Available
Enzyme 3 Locus Not Available
Enzyme 3 SNPs SNPJam Report Link Image
Enzyme 3 General References Not Available
Enzyme 3 Metabolite References Not Available
Enzyme 4 [top]
Enzyme 4 ID 12973
Enzyme 4 Name Glycine N-acyltransferase-like protein 2
Enzyme 4 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase-like protein 2
Enzyme 4 Gene Name GLYATL2
Enzyme 4 Protein Sequence >Glycine N-acyltransferase-like protein 2 
MLVLHNSQKLQILYKSLEKSIPESIKVYGAIFNIKDKNPFNMEVLVDAWPDYQIVITRPQ
KQEMKDDQDHYTNTYHIFTKAPDKLEEVLSYSNVISWEQTLQIQGCQEGLDEAIRKVATS
KSVQVDYMKTILFIPELPKKHKTSSNDKMELFEVDDDNKEGNFSNMFLDASHAGLVNEHW
AFGKNERSLKYIERCLQDFLGFGVLGPEGQLVSWIVMEQSCELRMGYTVPKYRHQGNMLQ
IGYHLEKYLSQKEIPFYFHVADNNEKSLQALNNLGFKICPCGWHQWKCTPKKYC
Enzyme 4 Number of Residues 294
Enzyme 4 Molecular Weight 34278
Enzyme 4 Theoretical pI 6.67
Enzyme 4 GO Classification Not Available
Enzyme 4 General Function Not Available
Enzyme 4 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 4 Pathways Not Available
Enzyme 4 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 4 Pfam Domain Function
Enzyme 4 Signals
  • None
Enzyme 4 Transmembrane Regions
  • None
Enzyme 4 Essentiality Not Available
Enzyme 4 GenBank ID Protein 29243559 Link Image
Enzyme 4 UniProtKB/Swiss-Prot ID Q8WU03 Link Image
Enzyme 4 UniProtKB/Swiss-Prot Entry Name GLYL2_HUMAN Link Image
Enzyme 4 PDB ID Not Available
Enzyme 4 Cellular Location Not Available
Enzyme 4 Gene Sequence Not Available
Enzyme 4 GenBank Gene ID AF426250 Link Image
Enzyme 4 GeneCard ID Q8WU03 Link Image
Enzyme 4 GenAtlas ID GLYATL2 Link Image
Enzyme 4 HGNC ID HGNC:24178 Link Image
Enzyme 4 Chromosome Location Not Available
Enzyme 4 Locus Not Available
Enzyme 4 SNPs SNPJam Report Link Image
Enzyme 4 General References Not Available
Enzyme 4 Metabolite References Not Available
Enzyme 5 [top]
Enzyme 5 ID 12974
Enzyme 5 Name Glycine N-acyltransferase-like protein 3
Enzyme 5 Synonyms Not Available
Enzyme 5 Gene Name GLYATL3
Enzyme 5 Protein Sequence >Glycine N-acyltransferase-like protein 3 
MLVLNCSTKLLILEKMLKSCFPESLKVYGAVMNINRGNPFQKEVVLDSWPDFKAVITRRQ
REAETDNLDHYTNAYAVFYKDVRAYRQLLEECDVFNWDQVFQIKGLQSELYDVSKAVANS
KQLNIKLTSFKAVHFSPVSSLPDTSFLKGPSPRLTYLSVANADLLNRTWSRGGNEQCLRY
IANLISCFPSVCVRDEKGNPVSWSITDQFATMCHGYTLPEHRRKGYSRLVALTLARKLQS
RGFPSQGNVLDDNTASISLLKSLHAEFLPCRFHRLILTPATFSGLPHL
Enzyme 5 Number of Residues 288
Enzyme 5 Molecular Weight 32704
Enzyme 5 Theoretical pI Not Available
Enzyme 5 GO Classification Not Available
Enzyme 5 General Function Not Available
Enzyme 5 Specific Function Acyltransferase which transfers the acyl group to the N-terminus of glycine (By similarity).
Enzyme 5 Pathways Not Available
Enzyme 5 Reactions Not Available
Enzyme 5 Pfam Domain Function Not Available
Enzyme 5 Signals
  • None
Enzyme 5 Transmembrane Regions
  • None
Enzyme 5 Essentiality Not Available
Enzyme 5 GenBank ID Protein Not Available
Enzyme 5 UniProtKB/Swiss-Prot ID Q5SZD4 Link Image
Enzyme 5 UniProtKB/Swiss-Prot Entry Name GLYL3_HUMAN Link Image
Enzyme 5 PDB ID Not Available
Enzyme 5 Cellular Location Not Available
Enzyme 5 Gene Sequence Not Available
Enzyme 5 GenBank Gene ID Not Available
Enzyme 5 GeneCard ID Q5SZD4 Link Image
Enzyme 5 GenAtlas ID GLYATL3 Link Image
Enzyme 5 HGNC ID HGNC:21349 Link Image
Enzyme 5 Chromosome Location Not Available
Enzyme 5 Locus Not Available
Enzyme 5 SNPs SNPJam Report Link Image
Enzyme 5 General References Not Available
Enzyme 5 Metabolite References Not Available