Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids

Duchenne muscular dystrophy (DMD) is an X-linked inherited disorder. Patients present with decreased bone mineral density (BMD) due to glucocorticoid therapy and progressive muscle weakness. Bone remodeling allows bone volume and structure to be maintained and controlled by local and systemic factor...

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Main Authors: Salvador Atilano-Miguel, Lourdes Barbosa-Cortés, Rocío Ortiz-Muñiz
Format: Article
Language:English
Published: Permanyer 2022-01-01
Series:Boletín Médico del Hospital Infantil de México
Subjects:
Online Access:https://www.bmhim.com/frame_esp.php?id=324
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author Salvador Atilano-Miguel
Lourdes Barbosa-Cortés
Rocío Ortiz-Muñiz
author_facet Salvador Atilano-Miguel
Lourdes Barbosa-Cortés
Rocío Ortiz-Muñiz
author_sort Salvador Atilano-Miguel
collection DOAJ
description Duchenne muscular dystrophy (DMD) is an X-linked inherited disorder. Patients present with decreased bone mineral density (BMD) due to glucocorticoid therapy and progressive muscle weakness. Bone remodeling allows bone volume and structure to be maintained and controlled by local and systemic factors. These include the receptor activator of the nuclear factor-kB (RANK)/RANK ligand (RANKL)/osteoprotegerin (OPG) system, a determining pathway in the balance between bone formation and resorption. Disruptions in this complex, caused by factors such as glucocorticoids, can affect bone metabolism. The extensive action of the RANK/RANKL/ OPG pathway suggests an influence on dystrophic muscle pathophysiology. This review aimed to highlight some aspects of the RANK/RANKL/OPG system, the effect of glucocorticoids on this pathway, and the pathophysiology of the patient with DMD.
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spelling doaj.art-80450c0b707b4192bb53e6922b9fd3e72022-12-22T03:26:06ZengPermanyerBoletín Médico del Hospital Infantil de México0539-61152022-01-0179510.24875/BMHIM.21000171Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoidsSalvador Atilano-Miguel0Lourdes Barbosa-Cortés1Rocío Ortiz-Muñiz2Unidad de Investigación Médica en Nutrición, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social; Doctorado en Ciencias Biológicas y de la Salud, Universidad Autónoma Metropolitana, Unidad Iztapalapa. Mexico City, MexicoUnidad de Investigación Médica en Nutrición, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, MexicoDepartamento de Ciencias de la Salud, División de Ciencias Biológicas y de la Salud, Universidad Autónoma Metropolitana, Unidad Iztapalapa, Mexico City, MexicoDuchenne muscular dystrophy (DMD) is an X-linked inherited disorder. Patients present with decreased bone mineral density (BMD) due to glucocorticoid therapy and progressive muscle weakness. Bone remodeling allows bone volume and structure to be maintained and controlled by local and systemic factors. These include the receptor activator of the nuclear factor-kB (RANK)/RANK ligand (RANKL)/osteoprotegerin (OPG) system, a determining pathway in the balance between bone formation and resorption. Disruptions in this complex, caused by factors such as glucocorticoids, can affect bone metabolism. The extensive action of the RANK/RANKL/ OPG pathway suggests an influence on dystrophic muscle pathophysiology. This review aimed to highlight some aspects of the RANK/RANKL/OPG system, the effect of glucocorticoids on this pathway, and the pathophysiology of the patient with DMD. https://www.bmhim.com/frame_esp.php?id=324Duchenne muscular dystrophy. RANK ligand. Osteoprotegerin (OPG). Receptor activator of nuclear factor-kappa B (RANKL). Glucocorticoids.
spellingShingle Salvador Atilano-Miguel
Lourdes Barbosa-Cortés
Rocío Ortiz-Muñiz
Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
Boletín Médico del Hospital Infantil de México
Duchenne muscular dystrophy. RANK ligand. Osteoprotegerin (OPG). Receptor activator of nuclear factor-kappa B (RANKL). Glucocorticoids.
title Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
title_full Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
title_fullStr Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
title_full_unstemmed Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
title_short Duchenne muscular dystrophy: RANK/RANKL/OPG (receptor activator of nuclear factor-kB/RANK ligand/osteoprotegerin) system and glucocorticoids
title_sort duchenne muscular dystrophy rank rankl opg receptor activator of nuclear factor kb rank ligand osteoprotegerin system and glucocorticoids
topic Duchenne muscular dystrophy. RANK ligand. Osteoprotegerin (OPG). Receptor activator of nuclear factor-kappa B (RANKL). Glucocorticoids.
url https://www.bmhim.com/frame_esp.php?id=324
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