Eating behaviour in contrasting adiposity phenotypes: Monogenic obesity and congenital generalized lipodystrophy

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dc.catalogadorgjm
dc.contributor.authorSantos Martín, José Luis
dc.contributor.authorCortés Mora, Víctor Antonio
dc.date.accessioned2024-01-19T14:48:18Z
dc.date.available2024-01-19T14:48:18Z
dc.date.issued2021
dc.description.abstractMost known types of nonsyndromic monogenic obesity are caused by rare mutations in genes of the leptin-melanocortin pathway controlling appetite and adiposity. In contrast, congenital generalized lipodystrophy represents the most extreme form of leanness in humans caused by recessive mutations in four genes involved in phospholipid/triglyceride synthesis and lipid droplet/caveolae structure. In this disease, the inability to store triglyceride in adipocytes results in hypoleptinemia and ectopic hepatic and muscle fat accumulation leading to fatty liver, hypertriglyceridemia and severe insulin resistance. As a result of hypoleptinemia, patients with lipodystrophy show alterations in eating behaviour characterized by constant increased energy intake. As it occurs in obesity caused by genetic leptin deficiency, exogenous leptin rapidly reduces hunger scores in patients with congenital generalized lipodystrophy, with additional beneficial effects on glucose homeostasis and metabolic profile normalization. The melanocortin-4 receptor agonist setmelanotide has been used in the treatment of monogenic obesities. There is only one report on the effect of setmelanotide in a patient with partial lipodystrophy resulting in mild reductions in hunger scores, with no improvements in metabolic status. The assessment of contrasting phenotypes of obesity/leanness represents an adequate strategy to understand the pathophysiology and altered eating behaviour associated with adipose tissue excessive accumulation/paucity.
dc.fechaingreso.objetodigital2024-12-12
dc.fuente.origenORCID
dc.identifier.doi10.1111/obr.13114
dc.identifier.urihttps://doi.org/10.1111/obr.13114
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/80694
dc.identifier.wosidWOS:000554414700001
dc.information.autorucEscuela de Medicina; Santos Martín, José Luis; 0000-0003-2895-0369; 1005923
dc.information.autorucEscuela de Medicina; Cortés Mora, Víctor Antonio; 0000-0002-1658-0965; 7576
dc.language.isoen
dc.nota.accesoContenido parcial
dc.rightsacceso restringido
dc.subjectLeanness
dc.subjectLeptin
dc.subjectLipodystrophy
dc.subjectObesity
dc.subject.ddc610
dc.subject.deweyMedicina y saludes_ES
dc.subject.ods02 Zero hunger
dc.subject.odspa02 Hambre cero
dc.titleEating behaviour in contrasting adiposity phenotypes: Monogenic obesity and congenital generalized lipodystrophy
dc.typeartículo
sipa.codpersvinculados1005923
sipa.codpersvinculados7576
sipa.trazabilidadORCID;2024-01-08
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