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Prior to secreting any of the bile acids (primary or secondary, see below), liver cells conjugate them with either glycine or taurine, to form a total of 8 possible conjugated bile acids. These conjugated bile acids are often referred to as bile salts. The pKa of the unconjugated bile acids are between 5 and 6.5, [4] and the pH of the duodenum ...
Finally, the conjugated bile acids which remained un-ionized conjugated bile acids are passively absorbed. Venous blood from the ileum goes straight into the portal vein and then into the liver sinusoids. There, hepatocytes extract bile acids very efficiently, and little escapes the healthy liver into systemic circulation.
Unlike deconjugation, bile acid reconjugation involves the addition of amino acids to an unconjugated bile acid. [7] Additionally, microbial bile acid conjugation is not limited to glycine or taurine. Instead, most amino acids can be conjugated to an unconjugated backbone. [7]
The bilirubin present in the plasma is largely unconjugated in this setting as they haven't been taken up and conjugated by the liver. [3] In this case, total serum bilirubin increases while the ratio of direct bilirubin to indirect bilirubin remains 96 to 4 as up to 96%-99% of bilirubin in the bile are conjugated mentioned above. [9] [1]
Bile consists of water, electrolytes, bile acids, cholesterol, phospholipids and conjugated bilirubin. [2] Some components are synthesized by hepatocytes (liver cells); the rest are extracted from the blood by the liver. [3] Bile is secreted by the liver into small ducts that join to form the common hepatic duct. [4]
In the liver, unconjugated bilirubin is up-taken by the hepatocytes and subsequently conjugated with glucuronic acid (via the enzyme uridine diphosphate–glucuronyl transferase). In this state, bilirubin is soluble in water and it is called conjugated bilirubin. [11] Conjugated bilirubin is excreted into the bile ducts and enters the duodenum.
Bilirubin metabolism. Haem is converted into unconjugated bilirubin then conjugated bilirubin. Conjugated bilirubin is then secreted along with bile into the intestine and is either excreted in faeces as urobilinogen or reabsorbed into blood and transported back to the liver.
Rotor syndrome (also known as Rotor type hyperbilirubinemia) [2] is a rare cause of mixed direct (conjugated) and indirect (unconjugated) hyperbilirubinemia, relatively benign, autosomal recessive [3] bilirubin disorder characterized by non-hemolytic jaundice due to the chronic elevation of predominantly conjugated bilirubin. [2]