He prohormone convertase mPC1 in AtT-20 cells. Mol Endocrinol. 1992; 6:1088?4. [PubMed: 1508222] 13. Hoshino A, Kowalska D, Jean F, et al. Modulation of PC1/3 activity by self-interaction and substrate binding. Endocrinology. 2011; 152:1402?1. [PubMed: 21303942] 14. Gradwohl G, Dierich A, LeMeur M, et al. neurogenin3 is expected for the development with the 4 endocrine cell lineages with the pancreas. Proc Natl Acad Sci USA. 2000; 97:1607?611. [PubMed: 10677506] 15. Creemers JWM, Choquet H, Stijnen P, et al. Heterozygous Mutations Causing Partial Prohormone Convertase 1 Deficiency Contribute To Human Obesity. Diabetes. 2012; 61:383?90. [PubMed: 22210313] 16. Henrich S, Lindberg I, Bode W, et al. Proprotein convertase models according to the crystal structures of furin and kexin: explanation of their specificity. J Mol Biol. 2005; 345:211?7. [PubMed: 15571716] 17. Zhou A, Bloomquist BT, Mains RE. The prohormone convertases PC1 and PC2 mediate distinct endoproteolytic cleavages inside a strict temporal order during proopiomelanocortin biosynthetic processing. J Biol Chem. 1993; 268:1763?. [PubMed: 8380577] 18. Creemers JW, Pritchard LE, Gyte A, et al. Agouti-related protein is posttranslationally cleaved by proprotein convertase 1 to produce agouti-related protein (AGRP)83-132: interaction between AGRP83-132 and melanocortin receptors can not be influenced by syndecan-3. Endocrinology. 2006; 147:1621?1. [PubMed: 16384863]NIH-PA Author Manuscript NIH-PA Author Manuscript NIH-PA Author ManuscriptGastroenterology. Author manuscript; accessible in PMC 2014 July 01.Mart et al.Page19. Brakch N, Rist B, Beck-Sickinger AG, et al. Function of prohormone convertases in pro-neuropeptide Y processing: coexpression and in vitro kinetic investigations. Biochemistry. 1997; 36:16309?0. [PubMed: 9405066] 20. Zhu X, Cao Y, Voogd K, et al. On the processing of proghrelin to ghrelin. J Biol Chem. 2006; 281:38867?0. [PubMed: 17050541] 21. Batterham RL, Cowley MA, Tiny CJ, et al. Gut hormone PYY(3-36) physiologically inhibits food intake. Nature. 2002; 418:650?. [PubMed: 12167864] 22. Boey D, Lin S, Karl T, et al. Peptide YY ablation in mice results in the improvement of hyperinsulinaemia and obesity. Diabetologia. 2006; 49:1360?0. [PubMed: 16680491] 23. Farooqi IS, Keogh JM, Yeo GS, et al. Clinical spectrum of obesity and mutations in the melanocortin four receptor gene.BuyGeranylgeraniol N Engl J Med.259214-55-6 structure 2003; 348:1085?five.PMID:35227773 [PubMed: 12646665] 24. Galanopoulou AS, Kent G, Rabbani SN, et al. Heterologous processing of prosomatostatin in constitutive and regulated secretory pathways. Putative role on the endoproteases furin, PC1, and PC2. J Biol Chem. 1993; 268:6041?. [PubMed: 8095501] 25. Elias CF. Leptin action in pubertal improvement: recent advances and unanswered inquiries. Trends Endocrinol Metab. 2012; 23:9?five. [PubMed: 21978495] 26. Dong W, Seidel B, Marcinkiewicz M, et al. Cellular localization of the prohormone convertases within the hypothalamic paraventricular and supraoptic nuclei. J Neurosci. 1997; 17:563?5. [PubMed: 8987779] 27. Sanchez VC, Goldstein J, Stuart RC, et al. Regulation of hypothalamic prohormone convertases 1 and two and effects on processing of prothyrotropin-releasing hormone. J Clin Invest. 2004; 114:357?9. [PubMed: 15286802]NIH-PA Author Manuscript NIH-PA Author Manuscript NIH-PA Author ManuscriptGastroenterology. Author manuscript; offered in PMC 2014 July 01.Mart et al.PageNIH-PA Author Manuscript NIH-PA Author ManuscriptFigure 1. Pedigrees and mutations in PCSKPedigrees.