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Inorganic pyrophosphate generation and disposition in pathophysiology
1Veterans Affairs Medical Center and School of Medicine, University of California, San Diego, California 92161, USA. rterkeltaub@ucsd.edu
American Journal of Physiology. Cell Physiology
|June 13, 2001
Summary
Inorganic pyrophosphate (PP(i)) is crucial for cell function and crystal deposition. Dysregulation of PP(i) is linked to diseases involving connective tissue calcification, highlighting its therapeutic potential.
Area of Science:
- Biochemistry
- Cell Biology
- Physiology
Background:
- Inorganic pyrophosphate (PP(i)) plays a role in intracellular processes and extracellular crystal formation.
- Cellular mechanisms for PP(i) production, degradation, and transport are specialized.
- Altered PP(i) metabolism is associated with various diseases and disease symptoms.
Purpose of the Study:
- To review recent advances in cellular PP(i) metabolism.
- To assess the physiological roles of intracellular PP(i) in higher organisms.
- To examine the link between extracellular PP(i) levels and connective tissue calcification disorders.
Main Methods:
- Literature review of recent molecular evidence.
- Analysis of the roles of ANK and PC-1 in PP(i) transport and generation.
- Assessment of PP(i) analogs in disease therapy.
Main Results:
- Decreased and increased extracellular PP(i) levels are directly linked to diseases with disordered matrix calcification.
- The transmembrane protein ANK regulates PP(i) movement between intracellular and extracellular compartments.
- The phosphodiesterase enzyme PC-1 generates PP(i) and is implicated in matrix calcification.
Conclusions:
- PP(i) metabolism is critical for maintaining connective tissue homeostasis.
- Understanding PP(i) pathways offers therapeutic strategies for calcification disorders.
- ANK and PC-1 are key regulators of PP(i) in matrix calcification.