Glycogen metabolism and glycogen storage diseases

F. Huijing

Research output: Contribution to journalReview articlepeer-review

44 Scopus citations


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 (US)
Pages (from-to)609-658
Number of pages50
JournalPhysiological Reviews
Issue number4
StatePublished - Jan 1 1975

ASJC Scopus subject areas

  • Physiology
  • Molecular Biology
  • Physiology (medical)


Dive into the research topics of 'Glycogen metabolism and glycogen storage diseases'. Together they form a unique fingerprint.

Cite this