| عنوان فارسی مقاله: | 2- اتیل هیدراکریلیک اسیدورا در نارسایی دهیدروژناز Acyl-CoA با زنجیره کوتاه: تشخیص و کاربردهای مسیر R در اکسیداسیون Isoleucine |
| عنوان انگلیسی مقاله: | 2-Ethylhydracrylic Aciduria in Short/Branched-Chain Acyl-CoA Dehydrogenase Deficiency: Application to Diagnosis and Implications for the R-Pathway of Isoleucine Oxidation |
بیماران و روشها
بیماران و ژنوتیپ
اسید آلی، اسید آمینه و تحلیل آکیل کارنیتن
اندازه گیری 2-MGB توسط GC-MS
تعیین Chiral اسید 2- متیل بوتیریک از GC-MS
نتایج
بحث
کلمات کلیدی :
Metabolome Analysis: An Introduction - Page 282 - Google Books Result https://books.google.com/books?isbn=047010550X Silas G. Villas-Boas, Jens Nielsen, Jorn Smedsgaard - 2007 - Science ... disease Prolinemia type II Propionic acidemia Propionic aciduria Purine nucleioside ... deficiency Sarcosinemia Short/branched chain acyl-CoA dehydrogenase ... Deoxyguanosine; Guanosine Sarcosine C5-Acylcarnitine; 2-Ethylhydracrylic ... ACYL CoA DEHYDROGENASE 9 DEFICIENCY - metagene.de www.metagene.de/disease/ACYLCoADEHYDROGENASE9DEFICIENCY_525.html 2-Ethylhydracrylic Aciduria in Short/Branched-Chain Acyl-CoA Dehydrogenase Deficiency: Application to Diagnosis and Implications for the R-Pathway of ... Human Metabolome Database: Showing metabocard for 2 ... www.hmdb.ca/metabolites/HMDB00396 Nov 16, 2005 - Showing metabocard for 2-Ethylhydracrylic acid (HMDB00396) ... 2-MBG) is the hallmark of short/branched-chain acyl-CoA dehydrogenase ... Purification and Characterization of 2-Methyl-branched Chain Acyl ... www.jbc.org/content/258/15/9477.full.pdf by Y Ikeda - 1983 - Cited by 114 - Related articles the dehydrogenation of all short branched chain acyl-CoAs. (1, 2). However, several years ago, our biochemical observa- tions on patients with isovaleric acidemia, an inborn error of .... to isolate crude 8-methyl-branched chain acyl-CoA dehydrogenase as ...... lites indicated that ethylhydracrylic aid was further oxidized.