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J Cell Biochem ; 118(12): 4762-4771, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-28523647

RESUMO

Mutations in the growth hormone receptor (GHR) gene can cause disruption of the growth hormone signaling pathway, resulting in growth deficiency due to growth hormone (GH) resistance. Both recessive and apparently dominant mutations have been described in the literature. In order to shed some light on the molecular mechanism of partial growth hormone resistance caused by heterozygous mutations, we performed an in-depth in silico analysis of a mutation found in a girl with a previous diagnosis of idiopathic short stature. An array of algorithms was used to predict pathogenicity and potential impact on the protein, and molecular modeling, docking and dynamics were used to determine structural consequences. The results suggest that both of the possible single mutation-containing heteromeric GH-GHR complexes, as well as the double GHR mutant complex result in perturbation of complex structures, with altered ability of the GHR dimers to interact with the GH peptide. J. Cell. Biochem. 118: 4762-4771, 2017. © 2017 Wiley Periodicals, Inc.


Assuntos
Proteínas de Transporte , Simulação por Computador , Transtornos do Crescimento , Heterozigoto , Mutação de Sentido Incorreto , Substituição de Aminoácidos , Proteínas de Transporte/genética , Proteínas de Transporte/metabolismo , Criança , Feminino , Transtornos do Crescimento/genética , Transtornos do Crescimento/metabolismo , Humanos
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