Journal
NEUROENDOCRINOLOGY
Volume 91, Issue 4, Pages 283-290Publisher
KARGER
DOI: 10.1159/000308880
Keywords
Prokineticins; GnRH secretion; Kallmann syndrome; Hypogonadotropic hypogonadism
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Funding
- Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [05/04726-0, 06/56753-3]
- Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPq) [300209/2008-8]
- Eunice Kennedy Shriver NICHD/NIH [U54 HD28138]
- EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH & HUMAN DEVELOPMENT [U54HD028138] Funding Source: NIH RePORTER
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The prokineticin system comprises two multifunctional secreted proteins, prokineticin-1 (PROK1) and prokineticin-2 (PROK2), and their cognate G protein-coupled receptors. The prokineticins were originally identified as endogenous regulators of gastrointestinal motility. Currently, these bioactive peptides are involved in a wide spectrum of biological functions, including angiogenesis, neurogenesis, circadian rhythms, nociception, hematopoiesis and immune response. Mice homozygous for null mutations in Prokr2 or Prok2 recapitulate the human phenotype of Kallmann syndrome, exhibiting severe atrophy of the reproductive system and hypoplastic olfactory bulbs. Indeed, the evidence from several naturally inactivating mutations in the PROK2 and PROKR2 genes in patients with Kallmann syndrome and normosmic hypogonadotropic hypogonadism also indicate the essential role of PROK2 in olfactory bulb morphogenesis and GnRH secretion in humans. Copyright (c) 2010 S. Karger AG, Basel
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