翻訳と辞書 Words near each other ・ Glycogen debranching enzyme ・ Glycogen phosphorylase ・ Glycogen phosphorylase isoenzyme BB ・ Glycogen storage disease ・ Glycogen storage disease type 0 ・ Glycogen storage disease type I ・ Glycogen storage disease type II ・ Glycogen storage disease type III ・ Glycogen storage disease type IV ・ Glycogen storage disease type IX ・ Glycogen storage disease type V ・ Glycogen storage disease type VI ・ Glycogen storage disease type XI ・ Glycogen synthase ・ Glycogen synthase kinase ・ Glycogen-branching enzyme deficiency ・ Glycogenase ・ Glycogenesis ・ Glycogenic acanthosis ・ Glycogenin ・ Glycogenin-1 ・ Glycogenolysis ・ Glycoinformatics ・ Glycol cleavage ・ Glycol dehydration ・ Glycol distearate ・ Glycol ethers ・ Glycol nucleic acid ・ Glycol stearate ・ Glycolaldehyde Dictionary Lists mini英和辞書 mini和英辞書 Webster 1913 Latin-English FOLDOC Wikipedia English ウィキペディア

翻訳と辞書　辞書検索 [ 開発暫定版 ] スポンサード リンク Glycogen-branching enzyme deficiency ： ウィキペディア英語版 Glycogen-branching enzyme deficiency Glycogen-branching enzyme deficiency (GBED) is an inheritable glycogen storage disease affecting American Quarter Horses and American Paint Horses. It leads to abortion, stillbirths, or early death of affected animals. ==Pathophysiology== Glycogen is a molecular polymer of glucose used to store energy. It is important for providing energy for skeletal and cardiac muscle contraction, and for maintaining glucose hemostasis in the blood. Molecules of glucose are linked into linear chains by α-1,4-glycosidic bonds. Additionally, branches of glucose are formed off of the chain via α-1,6-glycosidic bonds. 2 molecules of glucose are joined into an α-1,4-glycosidic bonds by an enzyme known as glycogen synthase. This bond may be broken by amylase when the body wishes to break down glycogen into glucose for energy. Glycogen branching enzyme is responsible for the required α-1,6-glycosidic bonds needed to start a branch off of these linear chains. These branches are important, as they provide additional "free ends" for linear chains of α-1,4-glycosidic bonds, which can then be broken down by amylase. This allows for glucose to be removed at a faster rate than if all glucose molecules were in a single chain with only two free ends on which amylase could attach. GBED is caused by an autosomal recessive mutation to the GBE1 gene, which leads glycogen branching enzyme activity that is reduced to absent.〔 Subsequently, glycogen molecules are produced with few branches, which greatly decreasing the number of nonreducing ends, drastically slowing the rate at which the molecule can be synthesized or broken down. This causes low levels of muscle glycogen that is very resistant to amylase. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Glycogen-branching enzyme deficiency」の詳細全文を読む スポンサード リンク 翻訳と辞書 : 翻訳のためのインターネットリソース Copyright(C) kotoba.ne.jp 1997-2016. All Rights Reserved.