Hers' disease

Hers' disease
also called  Glycogenosis Type Vi,  

      hereditary deficiency of the liver enzyme glycogen phosphorylase, which governs the metabolic breakdown of glycogen to the simple sugar glucose, which can then be used to meet the body's energy needs. The enzyme's absence causes glycogen to accumulate, greatly enlarging the liver and producing moderate hypoglycemia (low blood sugar), since the release of glucose from storage in the liver is impaired. Other symptoms of the disease are a mild increase in the acidity of the blood and growth retardation. No other organs seem to be involved. Unlike some of the related glycogen storage diseases, Hers' disease does not cause mental retardation or reduce the life span.

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Universalium. 2010.

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  • Hers disease — Hers disease. См. гликогеноз VI типа. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • Hers disease — (ārz) [Henri Gйry Hers, Belgian physiologist and biochemist, 20th century] glycogen storage disease, type VI; see under disease …   Medical dictionary

  • Hers disease — Glycogen storage disease in which there is a deficiency of liver phosphorylase …   Dictionary of molecular biology

  • Hers disease — glycogen storage d., type VI …   Medical dictionary

  • Disease — Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the… …   Medical dictionary

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  • Glycogen storage disease — Classification and external resources Glycogen ICD 10 E74.0 …   Wikipedia

  • Henri G. Hers — Henri Géry Hers (born 1923) is a Belgian physiologist and biochemist, and he was a professor at the Universite Catholique de Louvain. Hers disease is an inborn glycogen metabolism disorder caused by deficiency of hepatic phosphorylase, it is… …   Wikipedia

  • Glycogen storage disease type VI — Infobox Disease Name = PAGENAME Caption = Glycogen DiseasesDB = 5311 ICD10 = ICD10|E|74|0|e|70 ICD9 = ICD9|271.0 ICDO = OMIM = 232700 MedlinePlus = eMedicineSubj = med eMedicineTopic = 912 eMedicine mult = eMedicine2|ped|2564 | MeshID = D006013… …   Wikipedia

  • glycogen storage disease — Pathol. any of several inherited disorders of glycogen metabolism that result in excess accumulation of glycogen in various organs of the body. Also called glycogenosis /gluy koh jeuh noh sis/. * * * or glycogenosis Any of numerous types of… …   Universalium

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