WordNet

a part of a forked or branching shape; "he broke off one of the branches" (同)leg, ramification
divide into two or more branches so as to form a fork; "The road forks" (同)ramify, fork, furcate, separate
a division of a stem, or secondary stem arising from the main stem of a plant
a division of some larger or more complex organization; "a branch of Congress"; "botany is a branch of biology"; "the Germanic branch of Indo-European languages" (同)subdivision, arm
a stream or river connected to a larger one
any of several complex proteins that are produced by cells and act as catalysts in specific biochemical reactions

PrepTutorEJDIC

『枝』 / 『支流』,支脈,支線;分家 / 『支店』,『支部』,支局 / (学問の)『部門』,分科 / 枝を出す,枝を広げる / (川・道・鉄道などが)分岐する
〈U〉〈C〉(…の)(量・額などの)不足,欠乏《+『of』(『in』)+『名』》 / 〈C〉不足分,不足量,不足額 / 〈C〉(精神・肉体などの)欠陥
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1. アデノシンデアミナーゼ欠損症：治療 adenosine deaminase deficiency treatment
2. グルコースおよびグリコーゲン代謝の遺伝性疾患の概要 overview of inherited disorders of glucose and glycogen metabolism
3. 代謝性ミオパチーの原因 causes of metabolic myopathies
4. グリコーゲン分枝酵素欠損症（糖原病IV型、アンダーセン病） glycogen branching enzyme deficiency glycogen storage disease iv andersen disease
5. ホスホフルクトキナーゼ欠損症（糖原病VII型、垂井病） phosphofructokinase deficiency glycogen storage disease vii tarui disease

Diffuse reticuloendothelial system involvement in type IV glycogen storage disease with a novel GBE1 mutation: a case report and review.

Magoulas PL, El-Hattab AW, Roy A, Bali DS, Finegold MJ, Craigen WJ.SourceDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.
Human pathology.Hum Pathol.2012 Feb 1. [Epub ahead of print]
Glycogen storage disease type IV is a rare autosomal recessive disorder of glycogen metabolism caused by mutations in the GBE1 gene that encodes the 1,4-alpha-glucan-branching enzyme 1. Its clinical presentation is variable, with the most common form presenting in early childhood with primary hepati
PMID 22305237

Generation of a novel mouse model that recapitulates early and adult onset glycogenosis type IV.

Akman HO, Sheiko T, Tay SK, Finegold MJ, Dimauro S, Craigen WJ.SourceDepartment of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. hoakman@bcm.edu
Human molecular genetics.Hum Mol Genet.2011 Nov 15;20(22):4430-9. Epub 2011 Aug 19.
Glycogen storage disease type IV (GSD IV) is a rare autosomal recessive disorder caused by deficiency of the glycogen branching enzyme (GBE). The diagnostic feature of the disease is the accumulation of a poorly branched form of glycogen known as polyglucosan (PG). The disease is clinically heteroge
PMID 21856731

型	欠損酵素	組織	グリコーゲン構造	病名	発症時期	症状
I	グルコース-6-ホスファターゼ	肝臓	正常	フォン・ギールケ病	childhood	growth retardation, enlarged liver and kidney, hypoglycemia, elevated blood lactate, cholesterol, triglycerides, and uric acid
II	α-1,4-グルコシダーゼ	リソソーム	正常	ポンペ病	childhood
III	アミロ-1,6-グルコシダーゼ debrancher enzyme deficiency	全器官	最外層分子がないか非常に短い	コリ病	childhood	hypoglycemia, hepatomegaly, short-stature, and myopathty
IV	アミロ-(1,4→1,6)-トランスグリコシラーゼ brancher enzyme deficiency	肝臓、全器官?	非常に長い枝無し鎖	アンダーセン病		infantile failure to thrive, cirrhosis, liver failure, and extreme hypotonia.
V	グリコーゲンホスホリラーゼ muscle phosphorylase deficiency	筋肉	正常	マッカードル病	adult	exercise intolerance, muscle cramps, myoglobinuria on strenuous exercise, increased CK
VI	グリコーゲンホスホリラーゼ hepatic phosphorylase deficiency	肝臓	正常	エルス病		hematomegaly and variable hypoglycemia
VII	6-ホスホフルクト-1-キナーゼ	筋肉	正常	垂井病
VIII	ホスホリラーゼキナーゼ	肝臓	正常	X染色体ホスホリラーゼキナーゼ欠損症
IX	ホスホリラーゼキナーゼ	全器官	正常		childhoog
0	グリコーゲンシンターゼ	肝臓	正常だが少量