کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8259685 | 1534643 | 2015 | 9 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse: Urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency
ترجمه فارسی عنوان
مکانیسم افزایش سنتز گلیسرول کبدی در موشهای دوقلوی گلیسرول-3-فسفات دهیدروژناز گلیسرول / 3-فسفات دی هیدروژناز: گلیسرول ادرار و 3-فسفات گلیسرول به عنوان نشانگرهای تشخیصی کمبود الکل
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کلمات کلیدی
LACMGpdadult-onset type II citrullinemiaargininosuccinateαKGcitrin deficiencyPYRNeonatal intrahepatic cholestasis caused by citrin deficiencyNICCDCTLN2AGCGLNCITMCTALAASAPMSAOAASPDHAPG3Pα-ketoglutarate - α-کتوگلووتراتArginine - آرژنین aspartate - آسپارتاتalanine - آلانینAminooxyacetate - آمینوسیسی سکتهArg - ارگMedium-chain triglyceride - تری گلیسرید متوسط زنجیره ایRedox state - دولت Redoxdihydroxyacetone phosphate - دی هیدروکسی استون فسفاتCitrulline - سیترولینphenazine methosulfate - فنزین متوسولفاتLactate - لاکتاتknockout - ناکاوتwild-type - نوع وحشیPyruvate - پیرواتSodium pyruvate - پیروات سدیمcreatinine - کراتینینGlu - گلوglutamate - گلوتاماتglutamine - گلوتامینglycerol 3-phosphate - گلیسرول 3-فسفاتmitochondrial glycerol-3-phosphate dehydrogenase - گلیسرول 3-فسفات دهیدروژناز میتوکندریGlycerol - گلیسرول یا گلیسیرین
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
سالمندی
چکیده انگلیسی
The mitochondrial aspartate-glutamate carrier isoform 2 (citrin) and mitochondrial glycerol-3-phosphate dehydrogenase (mGPD) double-knockout mouse has been a useful model of human citrin deficiency. One of the most prominent findings has been markedly increased hepatic glycerol 3-phosphate (G3P) following oral administration of a sucrose solution. We aimed to investigate whether this change is detectable outside of the liver, and to explore the mechanism underlying the increased hepatic G3P in these mice. We measured G3P and its metabolite glycerol in plasma and urine of the mice under various conditions. Glycerol synthesis from fructose was also studied using the liver perfusion system. The citrin/mGPD double-knockout mice showed increased urine G3P and glycerol under normal, fed conditions. We also found increased plasma glycerol under fasted conditions, while oral administration of different carbohydrates or ethanol led to substantially increased plasma glycerol. Fructose infusion to the perfused liver of the double-knockout mice augmented hepatic glycerol synthesis, and was accompanied by a concomitant increase in the lactate/pyruvate (L/P) ratio. Co-infusion of either pyruvate or phenazine methosulfate, a cytosolic oxidant, with fructose corrected the high L/P ratio, leading to reduced glycerol synthesis. Overall, these findings suggest that hepatic glycerol synthesis is cytosolic NADH/NAD+ ratio-dependent and reveal a likely regulatory mechanism for hepatic glycerol synthesis following a high carbohydrate load in citrin-deficient patients. Therefore, urine G3P and glycerol may represent potential diagnostic markers for human citrin deficiency.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1852, Issue 9, September 2015, Pages 1787-1795
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1852, Issue 9, September 2015, Pages 1787-1795
نویسندگان
Mitsuaki Moriyama, Yuki Fujimoto, Shizuka Rikimaru, Miharu Ushikai, Eishi Kuroda, Kenji Kawabe, Katsura Takano, Akihiro Asakawa, Akio Inui, Kazuhiro Eto, Takashi Kadowaki, David S. Sinasac, Yoshiyuki Okano, Masahide Yazaki, Shu-ichi Ikeda, Chunhua Zhang,