THE MOUSE PALE EAR PIGMENT MUTANT AS A POSSIBLE ANIMAL-MODEL FOR HUMAN-PLATELET STORAGE POOL DEFICIENCY
- 1 January 1981
- journal article
- research article
- Vol. 57 (1) , 38-43
Abstract
The mouse pigment mutant pale ear, ep/ep, which has a defect in kidney lysosomal enzyme secretion, had prolonged bleeding on experimental injury. Platelet counts and platelet protein did not differ from normal. There was a deficiency in the platelet dense granule contents, serotonin, ATP and ADP. A marked reduction of platelet dense granules was observed by EM. Apparently pale ear is a useful animal model in the study of platelet storage pool disease. Studies on this mutant and other pigment mutants have established that 1 gene can regulate at least 3 subcellular organelles, including the melanosome, the lysosome and the platelet dense granule.This publication has 17 references indexed in Scilit:
- Synthesis and secretion of kidney beta-galactosidase in mutant le/le mice.Journal of Biological Chemistry, 1978
- Specific protease deficiency in polymorphonuclear leukocytes of Chédiak-Higashi syndrome and beige mice.The Journal of Experimental Medicine, 1978
- Abnormal Platelet Function in Chediak‐Higashi SyndromeBritish Journal of Haematology, 1977
- MEPACRINE, A TOOL FOR INVESTIGATING 5-HYDROXYTRYPTAMINE ORGANELLES OF BLOOD-PLATELETS BY FLUORESCENCE MICROSCOPY1977
- Correction of Leukocyte Function in Chediak-Higashi Syndrome by AscorbateNew England Journal of Medicine, 1976
- PLATELET ABNORMALITY IN CHEDIAK-HIGASHI-SYNDROME OF MAN1976
- PLATELET-FUNCTION IN CHEDIAK-HIGASHI-SYNDROME1976
- Simplified Determination of Blood Adenosine Triphosphate Using the Firefly SystemBlood, 1964
- PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENTJournal of Biological Chemistry, 1951
- Morphology and Enumeration of Human Blood PlateletsJournal of Applied Physiology, 1950