| neutral lipid storage disease | <syndrome> Congenital ichthyosis, leukocyte vacuoles, and variable involvement of other organ systems. Synonym: neutral lipid storage disease. (05 Mar 2000) |
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| disease, lipid storage | A series of disorders due to inborn errors in lipid metabolism resulting in the abnormal accumulation of lipids in the wrong places (Examples include Gaucher, Fabry and Niemann-Pick diseases and metachromatic leukodystrophy). (12 Dec 1998) |
| disease, phytanic acid storage | A genetic disorder of the fatty acid phytanic acid which accumulates and causes a number of progressive problems including polyneuritis (inflammation of numerous nerves), diminishing vision (due to retinitis pigmentosa), and wobbliness (ataxia) caused by damage to the cerebellar portion of the brain (cerebellar ataxia).(refsum's disease) (12 Dec 1998) |
| iron-storage disease | The storage of excess iron in the parenchyma of many organs, as in idiopathic haemochromatosis or transfusion haemosiderosis. (05 Mar 2000) |
| glycogen | <biochemistry> Branched polymer of D glucose (mostly _(1-4) linked, but some _(1-6) at branch points). Size range very variable, up to 10exp5 glucose units. Major short term storage polymer of animal cells and is particularly abundant in the liver and to a lesser extent in muscle. In the electron microscope glycogen has a characteristic asterisk or star appearance. (18 Nov 1997) |
| glycogen debranching enzyme system | 1,4-alpha-d-glucan-1,4-alpha-d-glucan 4-alpha-d-glucosyltransferase/dextrin 6 alpha-d-glucanohydrolase. An enzyme system having both 4-alpha-glucanotransferase (ec 2.4.1.25) and amylo-1,6-glucosidase (ec 3.2.1.33) activities. As a transferase it transfers a segment of a 1,4-alpha-d-glucan to a new 4-position in an acceptor, which may be glucose or another 1,4-alpha-d-glucan. As a glucosidase it catalyses the endohydrolysis of 1,6-alpha-d-glucoside linkages at points of branching in chains of 1,4-linked alpha-d-glucose residues. Amylo-1,6-glucosidase activity is deficient in glycogen storage disease type III. (12 Dec 1998) |
| glycogen granule | Glycogen occurring in cells as beta granule's which average about 300 A |
| glycogen phosphorylase | <enzyme> Enzyme that catalyses the sequential removal of glycosyl residues from glycogen to yield one glucose-1-phosphate per reaction. Its activity is controlled by phosphorylation (by phosphorylase kinase). (21 Jun 2000) |
| glycogen synthase | <enzyme> An enzyme of the transferase class that catalyses the reaction of udpglucose and (1,4-alpha-d-glucosyl)n to yield udp and 1,4-alpha-d-glucosyl)n+1. The reaction is highly regulated by allosteric effectors, by phosphorylation reactions, and by insulin. Chemical name: UDPglucose:glycogen 4-alpha-D-glucosyltransferase Registry number: EC 2.4.1.11 (12 Dec 1998) |
| glycogen synthase-d phosphatase | <enzyme> An enzyme that catalyses the conversion of phosphorylated, inactive glycogen synthase d to active dephosphoglycogen synthase I. Chemical name: (UDPglucose:glycogen 4-alpha-D-glucosyltransferase-D) phosphohydrolase Registry number: EC 3.1.3.42 (12 Dec 1998) |
| glycogen synthetase | <enzyme> An enzyme which makes amylose out of glucose molecules (a type of sugar) and attaches them to glycogen (a type of polysaccharide). (09 Oct 1997) |
| UDPglucose-glycogen glucose 1-phosphotransferase | <enzyme> Catalyses the transfer of glucose 1-phosphate onto c-6 of glucose residues in glycogen to form a phosphodiester Registry number: EC 2.7.8.- Synonym: ugg-glcptase (26 Jun 1999) |
| liver glycogen | Glycogen stored in the liver. (12 Dec 1998) |
| computer storage devices | Devices capable of receiving data, retaining data for an indefinite or finite period of time, and supplying data upon demand. (12 Dec 1998) |
| platelet storage pool deficiency | A group of disorders characterised by a decrease or lack of platelet dense bodies in which the releasable pool of adenine nucleotides and 5ht are normally stored. (12 Dec 1998) |