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Molecular pathology of glucose-6-phosphatase.

Abstract
It was known in the 1950s that hepatic microsomal glucose-6-phosphatase plays an important role in the regulation of blood glucose levels. All attempts since then to purify a single polypeptide with glucose-6-phosphatase activity have failed. Until recently, virtually nothing was known about the molecular basis of glucose-6-phosphatase or its regulation. Recent studies of the type 1 glycogen storage diseases, which are human genetic deficiencies that result in impaired glucose-6-phosphatase activity, have greatly increased our understanding of glucose-6-phosphatase. Glucose-6-phosphatase has been shown to comprise at least five different polypeptides, the catalytic subunit of glucose-6-phosphatase with its active site situated in the lumen of the endoplasmic reticulum; a regulatory Ca2+ binding protein; and three transport proteins, T1, T2, and T3, which respectively allow glucose-6-phosphate, phosphate, and glucose to cross the endoplasmic reticulum membrane. Purified glucose-6-phosphatase proteins, immunospecific antibodies, and improved assay techniques have led to the diagnosis of a variety of new type 1 glycogen storage diseases. Recent studies of the type 1 glycogen storage diseases have led to a much greater understanding of the role and regulation of each of the glucose-6-phosphatase proteins.
AuthorsA Burchell
JournalFASEB journal : official publication of the Federation of American Societies for Experimental Biology (FASEB J) Vol. 4 Issue 12 Pg. 2978-88 (Sep 1990) ISSN: 0892-6638 [Print] United States
PMID2168325 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Glucose-6-Phosphatase
  • Glucose
Topics
  • Biological Transport
  • Enzyme Activation (drug effects)
  • Glucose (metabolism)
  • Glucose-6-Phosphatase (analysis, isolation & purification, metabolism)
  • Glycogen Storage Disease Type I (classification, diagnosis, etiology)
  • Humans
  • Infant
  • Infant, Newborn
  • Microsomes, Liver (enzymology)
  • Protein Conformation
  • Sudden Infant Death (etiology)

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