WordNet

(physical chemistry) a sample of matter in which substances in different phases are in equilibrium; "in a static system oil cannot be replaced by water on a surface"; "a system generating hydrogen peroxide"
instrumentality that combines interrelated interacting artifacts designed to work as a coherent entity; "he bought a new stereo system"; "the system consists of a motor and a small computer"
a group of independent but interrelated elements comprising a unified whole; "a vast system of production and distribution and consumption keep the country going" (同)scheme
a complex of methods or rules governing behavior; "they have to operate under a system they oppose"; "that language has a complex system for indicating gender" (同)system of rules
a group of physiologically or anatomically related organs or parts; "the body has a system of organs for digestion"
a procedure or process for obtaining an objective; "they had to devise a system that did not depend on cooperation"
the living body considered as made up of interdependent components forming a unified whole; "exercise helped him get the alcohol out of his system"
one form in which body fuel is stored; stored primarily in the liver and broken down into glucose when needed by the body (同)animal_starch
resembling the branches of a tree
the act of branching out or dividing into branches (同)ramification, fork, forking
any of several complex proteins that are produced by cells and act as catalysts in specific biochemical reactions

PrepTutorEJDIC

〈C〉(関連した部分から成る)『体系』,系統,組織[網],装置 / 〈C〉(教育・政治などの)『制度』,機構;《the~》体制 / 〈C〉(思想・学問などの)『体系』,学説 / 〈C〉(…の)『方法』,方式,やり方《+of doing》 / 〈U〉正しい方針(筋道,順序) / 〈U〉《the~》(身体の)組織,系統 / 〈U〉《the~,one's~》身体,全身
グリコーゲン,糖原
酵素

UpToDate Contents

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1. 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
2. 心不全におけるアンギオテンシン変換酵素阻害剤およびアンギオテンシン受容体拮抗剤：作用機序 angiotensin converting enzyme inhibitors and receptor blockers in heart failure mechanisms of action
3. 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
4. Overview of the renin-angiotensin system
5. 高血圧治療におけるレニン・アンギオテンシン系阻害 renin angiotensin system inhibition in the treatment of hypertension

English Journal

Branching enzyme deficiency: expanding the clinical spectrum.

Paradas C1, Akman HO2, Ionete C3, Lau H4, Riskind PN3, Jones DE3, Smith TW5, Hirano M2, Dimauro S2.Author information 1Department of Neurology, Columbia University Medical Center, New York, New York2Unidad de Enfermedades Neuromusculares, Servicio de Neurología, Hospital Universitario Virgen del Rocío, Instituto de Biomédicina de Sevilla, Consejo Superior de Investigación.2Department of Neurology, Columbia University Medical Center, New York, New York.3Department of Neurology, University of Massachusetts Memorial Medical Center, Worcester.4Rusk Institute of Rehabilitation, NYU Langone Medical Center, New York, New York.5Department of Pathology, University of Massachusetts Memorial Medical Center, Worcester.AbstractIMPORTANCE: The neuromuscular presentation of glycogen branching enzyme deficiency includes a severe infantile form and a late-onset variant known as adult polyglucosan body disease. Herein, we describe 2 patients with adult acute onset of fluctuating neurological signs and brain magnetic resonance imaging lesions simulating multiple sclerosis. A better definition of this new clinical entity is needed to facilitate diagnosis.
JAMA neurology.JAMA Neurol.2014 Jan;71(1):41-7. doi: 10.1001/jamaneurol.2013.4888.
IMPORTANCE: The neuromuscular presentation of glycogen branching enzyme deficiency includes a severe infantile form and a late-onset variant known as adult polyglucosan body disease. Herein, we describe 2 patients with adult acute onset of fluctuating neurological signs and brain magnetic resonance
PMID 24248152

Cecropia peltata accumulates starch or soluble glycogen by differentially regulating starch biosynthetic genes.

Bischof S1, Umhang M, Eicke S, Streb S, Qi W, Zeeman SC.Author information 1Department of Biology, ETH Zurich, 8092 Zurich, Switzerland.AbstractThe branched glucans glycogen and starch are the most widespread storage carbohydrates in living organisms. The production of semicrystalline starch granules in plants is more complex than that of small, soluble glycogen particles in microbes and animals. However, the factors determining whether glycogen or starch is formed are not fully understood. The tropical tree Cecropia peltata is a rare example of an organism able to make either polymer type. Electron micrographs and quantitative measurements show that glycogen accumulates to very high levels in specialized myrmecophytic structures (Müllerian bodies), whereas starch accumulates in leaves. Compared with polymers comprising leaf starch, glycogen is more highly branched and has shorter branches--factors that prevent crystallization and explain its solubility. RNA sequencing and quantitative shotgun proteomics reveal that isoforms of all three classes of glucan biosynthetic enzyme (starch/glycogen synthases, branching enzymes, and debranching enzymes) are differentially expressed in Müllerian bodies and leaves, providing a system-wide view of the quantitative programming of storage carbohydrate metabolism. This work will prompt targeted analysis in model organisms and cross-species comparisons. Finally, as starch is the major carbohydrate used for food and industrial applications worldwide, these data provide a basis for manipulating starch biosynthesis in crops to synthesize tailor-made polyglucans.
The Plant cell.Plant Cell.2013 Apr;25(4):1400-15. doi: 10.1105/tpc.113.109793. Epub 2013 Apr 30.
The branched glucans glycogen and starch are the most widespread storage carbohydrates in living organisms. The production of semicrystalline starch granules in plants is more complex than that of small, soluble glycogen particles in microbes and animals. However, the factors determining whether gly
PMID 23632447

Starch modification with microbial alpha-glucanotransferase enzymes.

van der Maarel MJ1, Leemhuis H.Author information 1Food Innovation Center, AVEBE UA, Veendam, The Netherlands. m.j.e.c.van.der.maarel@rug.nlAbstractStarch is an agricultural raw material used in many food and industrial products. It is present in granules that vary in shape in the form of amylose and amylopectin. Starch-degrading enzymes are used on a large scale in the production of sweeteners (high fructose corn syrup) and concentrated glucose syrups as substrate for the fermentative production of bioethanol and basic chemicals. Over the last two decades α-glucanotransferases (EC 2.4.1.xx), such as branching enzyme (EC 2.4.1.18) and 4-α-glucanotransferase (EC 2.4.1.25), have received considerable attention. These enzymes do not hydrolyze the starch as amylases do. Instead, α-glucanotransferases remodel parts of the amylose and amylopectin molecules by cleaving and reforming α-1,4- and α-1,6-glycosidic bond. Here we review the properties of α-glucanotransferases and discuss the emerging use of these enzymes in the generation of novel starch derivatives.
Carbohydrate polymers.Carbohydr Polym.2013 Mar 1;93(1):116-21. doi: 10.1016/j.carbpol.2012.01.065. Epub 2012 Jan 31.
Starch is an agricultural raw material used in many food and industrial products. It is present in granules that vary in shape in the form of amylose and amylopectin. Starch-degrading enzymes are used on a large scale in the production of sweeteners (high fructose corn syrup) and concentrated glucos
PMID 23465909

Japanese Journal

A Note on the Metabolic System for the Synthesis of the Tandem-Cluster Structure of Amylopectin in Rice Endosperm

中村保典
Journal of Applied Glycoscience 51(3), 259-266, 2004
… The tandem-cluster structure of amylopectin must be a refined product that higher plants have evolved from the cluster-free random branched structure of glycogen. … During the evolutionary process, plants have increased the number of isozymes of three classes of enzymes, namely soluble starch synthase (SS), starch branching enzyme (BE) and starch debranching enzymes (DBE), by giving each individual enzyme a distinct function in constructing the amylopectin cluster structure. …
NAID 130004480832

Some Biochemical Aspects of the Biosynthesis of Glycogen and Amylopectin

Journal of applied glycoscience 50(4), 499-504, 2003-10-20
… Abstract: Whilst it is generally accepted that glycogen is synthesized from UDPG by the combined action of a chain-lengthening enzyme (glycogen synthase) and a branching enzyme (BE), the synthesis of amylopectinfrom ADPG requires a more complex enzyme system which includes various isoforms of the related starch synthase and plant BEs. …
NAID 10012469095

Histochemistry of Glycogen Synthesis in Tissue Cells

Proceedings of the Japanese Histochemical Association 1961(2), 98-110, 1961
… Histochemical mechanisms of intracellular glycogen synthesis, were discussed and two major pathways utilizing the amylophosphorylase→branching enzyme system and the UDPG-pyrophosphorylase→UDPG-glycogen transferase system were demonstrated histochemically. … The specificity of the histochemical demonstration of each enzyme and the methodology used in its demonstration were described. …
NAID 130004019783