Processing of proproteins to biologically active peptides and, in the case
of peptide hormones and neuropeptides, their sorting to granules of the reg
ulated secretory pathway, requires the concerted action of a cascade of enz
ymes and chaperones. The purpose of this review is to summarize the recent
emerging knowledge of how these molecules affect specific endocrine systems
. This has come about through the study of gene knockout mice as well as en
docrinopathies resulting from mutated genes in humans. (C) 1999 Elsevier Sc
ience Ireland Ltd. All rights reserved.