The α-granule proteome: novel proteins in normal and ghost granules in gray platelet syndrome.

ABSTRACT:

Background

Deficiencies in granule-bound substances in platelets cause congenital bleeding disorders known as storage pool deficiencies. For disorders such as gray platelet syndrome (GPS), in which thrombocytopenia, enlarged platelets and a paucity of α-granules are observed, only the clinical and histologic states have been defined.

Objectives

In order to understand the molecular defect in GPS, the α-granule fraction protein composition from a normal individual was compared with that of a GPS patient by mass spectrometry (MS).

Methods

Platelet organelles were separated by sucrose gradient ultracentrifugation. Proteins from sedimented fractions were separated by sodium dodecylsulfate polyacrylamide gel electrophoresis, reduced, alkylated, and digested with trypsin. Pe

SUBMITTER: Maynard DM 

PROVIDER: S-EPMC2953603 | biostudies-literature | 2010 Aug

REPOSITORIES: biostudies-literature