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Proprotein convertase-1/3 deficiency is an autosomal recessive disorder characterized by neonatal severe generalized malabsorptive diarrhea and failure to. Proprotein convertase 1/3 (pc1/3) deficiency, an autosomal-recessive disorder, results from homozygous mutations in the proprotein convertase subtilisin. Defective insulin processing is associated with obesity and diabetes. Prohormone convertase 1/3 (pc1/3) is an endopeptidase required for the. Pc1/3 deficiency is a very rare genetic disorder; thus far, only 21 patients have been reported (82, 83, 85–88, 90–93). It is very likely, however, that more. Proprotein convertase 1/3 (pc1/3) deficiency, an autosomal recessive disorder caused by rare mutations in the pcsk1 gene,. Prohormone convertase 1/3, encoded by the proprotein convertase subtilisin/kexin type 1 gene, is essential for processing prohormones;. Pc1/3 deficiency leads to severe watery diarrhea, impaired growth in infantile period, and severe obesity and endocrinopathies e. Proprotein convertase 1/3 (pc1/3) deficiency, an autosomal-recessive disorder caused by rare mutations in the proprotein convertase 
 
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