Cholic acid, also known as 3α,7α,12α-trihydroxy-5β-cholan-24-oic acid is a primary bile acid that is insoluble in water (soluble in alcohol and acetic acid), it is a white crystalline substance. Salts of cholic acid are called cholates. Cholic acid, along with

chenodeoxycholic acid Chenodeoxycholic acid (CDCA; also known as chenodesoxycholic acid, chenocholic acid and 3α,7α-dihydroxy-5β-cholan-24-oic acid) is a bile acid. Salts of this carboxylic acid are called chenodeoxycholates. Chenodeoxycholic acid is one of the mai ...

, is one of the two major bile acids produced by the

liver The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...

, where it is synthesized from

cholesterol Cholesterol is any of a class of certain organic molecules called lipids. It is a sterol (or modified steroid), a type of lipid. Cholesterol is biosynthesized by all animal cells and is an essential structural component of animal cell mem ...

. These two major bile acids are roughly equal in concentration in humans. Derivatives are made from cholyl-CoA, which exchanges its CoA with either

glycine Glycine (symbol Gly or G; ) is an amino acid that has a single hydrogen atom as its side chain. It is the simplest stable amino acid ( carbamic acid is unstable), with the chemical formula NH2‐ CH2‐ COOH. Glycine is one of the proteinog ...

, or

taurine Taurine (), or 2-aminoethanesulfonic acid, is an organic compound that is widely distributed in animal tissues. It is a major constituent of bile and can be found in the large intestine, and accounts for up to 0.1% of total human body weight. I ...

, yielding glycocholic and

taurocholic acid Taurocholic acid, known also as cholaic acid, cholyltaurine, or acidum cholatauricum, is a deliquescent yellowish crystalline bile acid involved in the emulsification of fats. It occurs as a sodium salt in the bile of mammals. It is a conjugat ...

, respectively. Cholic acid downregulates

cholesterol-7-α-hydroxylase Cholesterol 7 alpha-hydroxylase also known as cholesterol 7-alpha-monooxygenase or cytochrome P450 7A1 (CYP7A1) is an enzyme that in humans is encoded by the gene which has an important role in cholesterol metabolism. It is a cytochrome P450 enz ...

(rate-limiting step in bile acid synthesis), and cholesterol does the opposite. This is why chenodeoxycholic acid, and not cholic acid, can be used to treat

gallstone A gallstone is a stone formed within the gallbladder from precipitated bile components. The term cholelithiasis may refer to the presence of gallstones or to any disease caused by gallstones, and choledocholithiasis refers to the presence of mi ...

s (because decreasing bile acid synthesis would supersaturate the stones even more). Cholic acid and

are the most important human bile acids. Other species may synthesize different bile acids as their predominant primary bile acids.

Medical uses

Cholic acid, sold under the brand name Cholbam, is approved for use in the United States and is indicated as a treatment for children and adults with bile acid synthesis disorders due to single enzyme defects, and for peroxisomal disorders (such as

Zellweger syndrome Zellweger syndrome is a rare congenital disorder characterized by the reduction or absence of functional peroxisomes in the cells of an individual. It is one of a family of disorders called Zellweger spectrum disorders which are leukodystrophies ...

). It was approved for use in the European Union in September 2013, and is sold under the brand name Orphacol. Text was copied from this source which is under copyright of the European Medicines Agency. Reproduction is authorized provided the source is acknowledged. It is indicated for the treatment of inborn errors in primary bile-acid synthesis due to 3β-hydroxy-Δ⁵-C₂₇-steroid oxidoreductase deficiency or Δ⁴-3-oxosteroid-5β-reductase deficiency in infants, children and adolescents aged one month to 18 years and adults. Cholic acid FGK (Kolbam) was approved for medical use in the European Union in November 2015. Text was copied from this source which is under copyright of the European Medicines Agency. Reproduction is authorized provided the source is acknowledged. It is indicated for the treatment of inborn errors of primary bile acid synthesis, in infants from one month of age for continuous lifelong treatment through adulthood, encompassing the following single enzyme defects: *

sterol 27-hydroxylase CYP27A1 is a gene encoding a cytochrome P450 oxidase, and is commonly known as sterol 27-hydroxylase. This enzyme is located in many different tissues where it is found within the mitochondria. It is most prominently involved in the biosynthesis ...

deficiency (presenting as

cerebrotendinous xanthomatosis Cerebrotendinous xanthomatosis, also called cerebral cholesterosis, is an autosome, autosomal dominance (genetics), recessive form of xanthomatosis. It falls within a group of genetic disorders called the Leukodystrophy, leukodystrophies. Presenta ...

, CTX); * 2- (or alpha-) methylacyl-CoA racemase (AMACR) deficiency; * cholesterol 7 alpha-hydroxylase (CYP7A1) deficiency. The most common side effects include peripheral neuropathy (nerve damage in the hands and feet), diarrhea, nausea (feeling sick), acid reflux (stomach acid flowing up into the mouth), esophagitis (inflammation of the food pipe), jaundice (yellowing of the skin and eyes), skin problems (lesions) and malaise (feeling unwell). Cholic Acid vs Other Bile Acids

Interactive pathway map

References

External links

* {{DEFAULTSORT:Cholic Acid Bile acids Triols Cholanes Orphan drugs