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Review

Hyperphagia and Obesity in Prader–Willi Syndrome: PCSK1 Deficiency and Beyond?

1
Laboratory of Cellular and Molecular Endocrinology, Institute of Biomedical Research in Malaga (IBIMA), Virgen de la Victoria University Hospital, 29010 Malaga, Spain
2
Department of Endocrinology, Virgen de la Victoria University Hospital, 29010 Malaga, Spain
3
CIBER Fisiopatología de la Obesidad y Nutrición (CIBERObn CB06/003), Instituto de Salud Carlos III, 28029 Madrid, Spain
4
Laboratory of Biochemical Neuroendocrinology, Department of Human Genetics, KU Leuven, B-3000 Leuven, Belgium
*
Author to whom correspondence should be addressed.
These authors contributed equally.
Genes 2018, 9(6), 288; https://doi.org/10.3390/genes9060288
Received: 9 May 2018 / Revised: 4 June 2018 / Accepted: 4 June 2018 / Published: 7 June 2018
(This article belongs to the Special Issue Advances in Genetics of Regeneration in Metabesity)
Prader–Willi syndrome (PWS) is a complex genetic disorder that, besides cognitive impairments, is characterized by hyperphagia, obesity, hypogonadism, and growth impairment. Proprotein convertase subtilisin/kexin type 1 (PCSK1) deficiency, a rare recessive congenital disorder, partially overlaps phenotypically with PWS, but both genetic disorders show clear dissimilarities as well. The recent observation that PCSK1 is downregulated in a model of human PWS suggests that overlapping pathways are affected. In this review we will not only discuss the mechanisms by which PWS and PCSK1 deficiency could lead to hyperphagia but also the therapeutic interventions to treat obesity in both genetic disorders. View Full-Text
Keywords: Prader–Willi syndrome; PCSK1 deficiency; obesity; hyperphagia; hypothalamus Prader–Willi syndrome; PCSK1 deficiency; obesity; hyperphagia; hypothalamus
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MDPI and ACS Style

Ramos-Molina, B.; Molina-Vega, M.; Fernández-García, J.C.; Creemers, J.W. Hyperphagia and Obesity in Prader–Willi Syndrome: PCSK1 Deficiency and Beyond? Genes 2018, 9, 288. https://doi.org/10.3390/genes9060288

AMA Style

Ramos-Molina B, Molina-Vega M, Fernández-García JC, Creemers JW. Hyperphagia and Obesity in Prader–Willi Syndrome: PCSK1 Deficiency and Beyond? Genes. 2018; 9(6):288. https://doi.org/10.3390/genes9060288

Chicago/Turabian Style

Ramos-Molina, Bruno, María Molina-Vega, José C. Fernández-García, and John W. Creemers 2018. "Hyperphagia and Obesity in Prader–Willi Syndrome: PCSK1 Deficiency and Beyond?" Genes 9, no. 6: 288. https://doi.org/10.3390/genes9060288

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