Storage pool diseases illuminate platelet dense granule biogenesis
نویسندگان
چکیده
منابع مشابه
Dense-core secretory granule biogenesis.
The dense-core secretory granule is a key organelle for secretion of hormones and neuropeptides in endocrine cells and neurons, in response to stimulation. Cholesterol and granins are critical for the assembly of these organelles at the trans-Golgi network, and their biogenesis is regulated quantitatively by posttranscriptional and posttranslational mechanisms.
متن کاملQuantification of a novel dense granule protein (granulophysin) in platelets of patients with dense granule storage pool deficiency.
An antigen-capture sandwich enzyme-linked immunosorbent assay (ELISA) was developed for a novel protein granulophysin, a constituent of the platelet dense granule (DG) membrane and used to characterize patients with dense granule storage pool deficiency (delta-SPD). The assay uses two monoclonal antibodies against the protein, one of which is conjugated to peroxidase. Purified DGs, an enriched ...
متن کاملThrombin and ionophore A23187-induced dense granule secretion in storage pool deficient platelets: evidence for impaired nucleotide storage as the primary dense granule defect.
The secretion of the dense granule constituents ATP, ADP, calcium, pyrophosphate (PPi), and orthophosphate (Pi), and the release of magnesium induced by thrombin and the divalent cation ionophore A23187 have been quantitated directly in gel-filtered platelets from patients with storage pool deficiency (SPD). Both the contents and the maximal amounts of the dense granule constituents secretable ...
متن کاملThrombin and lonophore A23187-Induced Dense Granule Secretion in Storage Pool Deficient Platelets: Evidence for Impaired Nucleotide Storage as the Primary Dense Granule Defect
The secretion of the dense granule constituents ATP. ADP, calcium. pyrophosphate (PP,). and orthophosphate (PJ. and the release of magnesium induced by thrombin and the divalent cation ionophore A231 87 have been quantitated directly in gel-filtered platelets from patients with storage pool deficiency (SPD). Both the contents and the maximal amounts of the dense granule constituents secretable ...
متن کاملPlatelet storage pool deficiency in pigs.
We report a new bleeding disease--storage pool deficiency (SPD) of platelets--in pigs from the Mayo swine colony of homozygous von Willebrand's disease (vWD) and of heterozygous carriers of vWD. Levels of factor VIII, von Willebrand factor antigen (vWF:Ag), and ristocetin cofactor (RCof) were similar in the vWD carriers and SPD pigs. The latter pigs, however, had bleeding times of 15 minutes or...
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ژورنال
عنوان ژورنال: Platelets
سال: 2016
ISSN: 0953-7104,1369-1635
DOI: 10.1080/09537104.2016.1243789