20 years of leptin: human disorders of leptin action

J Endocrinol. 2014 Oct;223(1):T63-70. doi: 10.1530/JOE-14-0480.

Abstract

The discovery of leptin has provided a robust framework upon which our current understanding of the mechanisms involved in energy homeostasis has been built. In this review, we describe how the identification of humans with mutations in the genes encoding leptin and the leptin receptor and the characterisation of the associated clinical phenotypes have provided insights into the role of leptin-responsive pathways in the regulation of eating behaviour, intermediary metabolism and the onset of puberty. Importantly, administration of recombinant human leptin in leptin deficiency represents the first mechanistically based targeted therapy for obesity and has provided immense clinical benefits for the patients concerned. In subsequent years, we and others have shown that human obesity can result from a multiplicity of defects in the pathways downstream of leptin signalling within the brain.

Keywords: leptin; obesity; receptors; signal transduction.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Brain / drug effects
  • Brain / metabolism
  • Brain / physiopathology
  • Energy Metabolism / drug effects
  • Energy Metabolism / genetics
  • Energy Metabolism / physiology
  • Humans
  • Leptin / deficiency
  • Leptin / genetics*
  • Leptin / therapeutic use
  • Models, Genetic
  • Mutation*
  • Obesity / drug therapy
  • Obesity / genetics*
  • Obesity / metabolism
  • Receptors, Leptin / genetics
  • Receptors, Leptin / metabolism
  • Signal Transduction / drug effects
  • Signal Transduction / genetics*

Substances

  • Leptin
  • Receptors, Leptin