Inorganic pyrophosphate levels in blood platelets from normal donors and patients with calcium pyrophosphate dihydrate crystal deposition disease

✍ Scribed by Lawrence M. Ryan; Michael P. Lynch; Daniel J. Mccarty

• Publisher: John Wiley and Sons
• Year: 1983
• Tongue: English
• Weight: 251 KB
• Volume: 26
• Category: Article
• ISSN: 0004-3591
• DOI: 10.1002/art.1780260419

✦ Synopsis

We measured the intracellular pyrophosphate level of platelets from patients with calcium pyrophosphate dihydrate deposition disease. Total and secretory granule pyrophosphates were in the same range in patients with chondrocalcinosis and in normal volunteers. Therefore, not all cells share in the elevated levels previously reported for cultured cells from a kindred with familial chondrocalcinosis. An unexpected correlation between age of the donor and platelet pyrophosphate content was noted.

Lust et al reported elevated intracellular inorganic pyrophosphate (PPi) in cultured fibroblasts and lymphoblasts obtained from affected members of a French kindred with familial calcium pyrophosphate dihydrate JCPPD) crystal deposition (1,2). The PPi contents of cells cultured from nonaffected family members and normal donors were similar and consistently lower than levels in cells from affected family members. Thus, intracellular PPi may, in certain patients, be a biochemical disease marker. Platelets are rich in PPi; upon stimulation, they secrete this substance with a time course that parallels release of adeniqe nucleotides (3). They are more readily obtained and prepared than cultured cells. We compared