Glycogen metabolism and glycogen storage diseases

F. Huijing

Research output: Contribution to journalArticle

44 Citations (Scopus)

Abstract

Biochemists, physiologists, and pharmacologists have studied glycogen and glycogen metabolism for over 100 years. These studies have yielded data of great significance and applicability to the whole of physiological chemistry, including the realization that hormones alter enzyme activities and thus regulate metabolism, the detection of cyclic AMP and the concept of lysosomal storage diseases. Glycogen was the first biopolymer to be synthesized in vitro. Genetic defects of glycogen metabolism have been known in man since the early 1920's and lately have also been found in other vertebrates. Recently, it has become possible to assign at least two enzymes of glycogen metabolism to specific chromosomes. At the molecular level, it has become apparent that regulatory sites of two enzymes of glycogen metabolism show a remarkable homology of sequence, indicating a common ancestry of at least these regulatory parts of these molecules. Studies of glycogen are still yielding important new information and the glycogen storage diseases provide methods to check information obtained in studies on isolated enzymes, tissue extracts, or whole organisms. Before receiving the glycogen storage diseases, the known facts on glycogen structure, metabolism and control of metabolism are summarized, emphasizing the new developments in this field.

Original languageEnglish
Pages (from-to)609-658
Number of pages50
JournalPhysiological Reviews
Volume55
Issue number4
StatePublished - Dec 1 1975
Externally publishedYes

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Glycogen Storage Disease
Glycogen
Enzymes
Lysosomal Storage Diseases
Biopolymers
Tissue Extracts
Information Storage and Retrieval
Sequence Homology
Cyclic AMP
Vertebrates
Chromosomes
Hormones

ASJC Scopus subject areas

  • Physiology

Cite this

Glycogen metabolism and glycogen storage diseases. / Huijing, F.

In: Physiological Reviews, Vol. 55, No. 4, 01.12.1975, p. 609-658.

Research output: Contribution to journalArticle

Huijing, F 1975, 'Glycogen metabolism and glycogen storage diseases', Physiological Reviews, vol. 55, no. 4, pp. 609-658.
Huijing, F. / Glycogen metabolism and glycogen storage diseases. In: Physiological Reviews. 1975 ; Vol. 55, No. 4. pp. 609-658.
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