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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
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Home Page.
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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