کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
5736891 | 1411964 | 2017 | 34 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Suppression of brain cholesterol synthesis in male Mecp2-deficient mice is age dependent and not accompanied by a concurrent change in the rate of fatty acid synthesis
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کلمات کلیدی
SQLEHMGCRSC4MOLMecp224S-OHCSqualene monooxygenase24(S)-HydroxycholesterolCYP27A1methyl CpG-binding protein 2CYP46A13-hydroxy-3-methylglutaryl-CoA reductase - 3-هیدروکسی-3-methylglutaryl-CoA ردوکتازApoe - آپوapolipoprotein E - آپولیپوپروتئین EOntogeny - انتگرالCNS - دستگاه عصبی مرکزیDesmosterol - دسمسترولFatty acid synthesis - سنتز اسید چربRett syndrome - سندروم رتcentral nervous system - سیستم عصبی مرکزیbrain weight - وزن مغزCholesterol 24-hydroxylase - کلسترول 24-هیدروکسیلاز
موضوعات مرتبط
علوم زیستی و بیوفناوری
علم عصب شناسی
علوم اعصاب (عمومی)
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
Mutations in the X-linked gene methyl-CpG-binding protein 2 (MECP2) are the principal cause of Rett syndrome, a progressive neurodevelopmental disorder afflicting 1 in 10,000 to 15,000 females. Studies using hemizygous Mecp2 mouse models have revealed disruptions to some aspects of their lipid metabolism including a partial suppression of cholesterol synthesis in the brains of mature Mecp2 mutants. The present studies investigated whether this suppression is evident from early neonatal life, or becomes manifest at a later stage of development. We measured the rate of cholesterol synthesis, in vivo, in the brains of male Mecp2â/y and their Mecp2+/y littermates at 7, 14, 21, 28, 42 and 56 days of age. Brain weight was consistently lower in the Mecp2â/y mice than in their Mecp2+/y controls except at 7 days of age. In the 7- and 14-day-old mice there was no genotypic difference in the rate of brain cholesterol synthesis but, from 21 days and later, it was always marginally lower in the Mecp2â/y mice than in age-matched Mecp2+/y littermates. At no age was a genotypic difference detected in either the rate of fatty acid synthesis or cholesterol concentration in the brain. Cholesterol synthesis rates in the liver and lungs of 56-day-old Mecp2â/y mice were normal. The onset of lower rates of brain cholesterol synthesis at about the time closure of the blood brain barrier purportedly occurs might signify a disruption to mechanism(s) that dictate intracellular levels of cholesterol metabolites including oxysterols known to exert a regulatory influence on the cholesterol biosynthetic pathway.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Brain Research - Volume 1654, Part A, 1 January 2017, Pages 77-84
Journal: Brain Research - Volume 1654, Part A, 1 January 2017, Pages 77-84
نویسندگان
Adam M. Lopez, Jen-Chieh Chuang, Kenneth S. Posey, Stephen D. Turley,