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Proenzyme Convertase 1


Proenzyme convertase 1 (PC-1) cuts a part off of proopiomelanocortin (PMOC). This part it produces is a protein called a-MSH which inhibits appetite by binding to the MC4R receptor in the brain. If there is a mutation in the proenzyme convertase 1 gene, then a-MSH cannot be produced from PMOC. PC-1 also helps to produce insulin and glucagon.

Children with this mutation eat large amounts of food. They can weigh more than 77 lbs by 3 years old. POMC is made into ACTH which signals the production of cortisol. Because of this, PC-1 deficiency causes low cortisol levels. Glucagon stimulates the lining of the small intestine to grow. Less glucagon leads to decreased absorbing capacity of the small intestine. Only 3 cases have been reported.

The hypothalamic leptin-melanocortin system
The hypothalamic leptin-melanocortin system

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It is important you discuss any weight loss or exercise plan with your doctor. Only you and your physician can decide what is best for you. Some people have certain conditions that prevent them from doing all exercises, and goal body weights may be different for different people. You need to discuss all these things with your physician before starting any weight loss or exercise program.

This article was written by John Vickery, MD.

References

Fauci et al., Harrison's Principles of Internal Medicine 17th ed., ch 74
Endocrinol Metab Clin N Am 2008;37:733-751

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