Storage pool disease: comparative fluorescence microscopical, cytochemical and biochemical studies on amine-storing organelles of human blood platelets.
The platelet content of ATP, ADP, serotonin (5-HT), dopamine, noradrenaline and adrenaline as well as the net uptake of radiolabelled 5-HT and mepacrine were subnormal in six patients with Storage Pool Disease (SPD). Fewer amine-storing organelles were found by fluorescence microscopy with the fluorescent probe mepacrine and by electron microscopy with a cytochemical (uranaffin) reaction specific for 5'-phosphonucleotides. Both methods showed that SPD platelets have atypical organelles with a reduced capacity to store amines, 5'-phosphonucleotides and mepacrine. The changes were most marked in platelets of the two patients who also had oculocutaneous albinism.
D000244:Adenosine Diphosphate / Q000097:blood; D000255:Adenosine Triphosphate / Q000097:blood; D000293:Adolescent; D000328:Adult; D001791:Blood Platelet Disorders / Q000097:blood / Q000235:genetics*; D001792:Blood Platelets / Q000378:metabolism* / Q000648:ultrastructure; D002395:Catecholamines / Q000097:blood; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009940:Organoids / Q000378:metabolism* / Q000648:ultrastructure; D011796:Quinacrine / Q000097:blood; D012701:Serotonin / Q000097:blood*
D002395:Catecholamines; D012701:Serotonin; D000244:Adenosine Diphosphate; D000255:Adenosine Triphosphate; D011796:Quinacrine