CHOLBAM ™

Mechanism of Action
Cholic acid is a primary bile acid synthesized from cholesterol in the liver. In bile acid synthesis disorders due to SEDs in the biosynthetic pathway, and in PDs including Zellweger spectrum disorders, deficiency of primary bile acids leads to unregulated accumulation of intermediate bile acids and cholestasis. Bile acids facilitate fat digestion and absorption by forming mixed micelles, and facilitate absorption of fat-soluble vitamins in the intestine.

Endogenous bile acids including cholic acid enhance bile flow and provide the physiologic feedback inhibition of bile acid synthesis. The mechanism of action of cholic acid has not been fully established; however, it is known that cholic acid and its conjugates are endogenous ligands of the nuclear receptor, farnesoid X receptor (FXR). FXR regulates enzymes and transporters that are involved in bile acid synthesis and in the enterohepatic circulation to maintain bile acid homeostasis under normal physiologic conditions.

INDICATIONS AND USAGE:
CHOLBAM is a bile acid indicated for:
Treatment of bile acid synthesis disorders due to single enzyme defects (SEDs).
Adjunctive treatment of peroxisomal disorders (PDs) including Zellweger spectrum disorders in patients who exhibit manifestations of liver disease, steatorrhea or complications from decreased fat soluble vitamin absorption

Limitation of use:
The safety and effectiveness of CHOLBAM on extrahepatic manifestations of bile acid synthesis disorders due to SEDs or PDs including Zellweger spectrum disorders have not been established.

CHOLBAM capsules are available as two-piece gelatin capsules with a Swedish orange cap imprinted with "50mg" and Swedish orange body with imprinted with "ASK001". The capsules contain a white or off-white powder and are supplied in bottles of:

90 capsules (NDC 45043-001-02)
250 mg Capsules

CHOLBAM capsules are available as two-piece gelatin capsules with a white cap imprinted with "250mg" and white body with imprinted with "ASK002". The capsules contain a white or off-white powder and are supplied in bottles of:

90 capsules (NDC 45043-002-02)
Storage and Handling

Store at 20–25°C (69-77°F), excursions permitted between 15-30°C (59-86°F). [see USP Controlled Room Temperature].