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In agreement with this, mutations in PC 1/3 lead to abnormalities in GLP-1 processing and secretion, associated with multiple endocrinopathies (143) (undoubtedly because PC 1/3 is important for processing of many regulatory peptides/hormones), and mice with a targeted deletion of the PC-1/3 gene are unable to process proglucagon to GLP-2 and GLP-1 (295, 344)

Seek medical attention promptly for severe abdominal pain, repeated vomiting, trouble breathing, swelling of the face or throat, severe dehydration, or symptoms of low blood sugar

Each bar for qRT-PCR data represents the mean value of three technically repeated samples and each qRT-PCR experiment was repeated once (same for other qRT-PCR data in this paper)

Acknowledgements PSA is supported by the American College of Medical Genetics Foundation/Genzyme 2014-2015 Fellowship in Biochemical Genetics

This rise is usually observed within weeks of therapy initiation

L(-)-carnitine production using a recombinant Escherichia coli strain