Hereditary spherocytosis (HS) is the most common red cell membrane defect resulting from
protein abnormalities. However, changes in red
cell membrane proteins in HS remain under-investigated. We therefore evaluated red cell membrane
proteome in non-splenectomized, mild-degree HS patients (n = 9) compared to healthy individuals (n = 5).
Proteins derived from the red cell membranes of each subject were resolved in each two-dimensional gel and visualized by Deep Purple fluorescence staining. Spot matching and quantitative intensity analysis revealed 56 differentially expressed
protein spots (41 increased and 15 decreased), which were then successfully identified by quadrupole time-of-flight mass spectrometry. Among these, seven
isoforms/subunits of
spectrin were markedly increased (up to 10.51 folds), whereas two
isoforms/subunits of band-3
protein were decreased approximately 50% as compared to normal red cells. However, two
isoforms/subunits of
protein 4.1 were increased, while another
isoform/subunit was decreased. All these significantly altered
proteins were subjected to global
protein network analysis using Ingenuity Pathways Analysis tool, which revealed three important networks related to HS, including Network I: Cell death, genetic and hematological disorders; Network II: Cell cycle, carbohydrate metabolism and molecular transport; and Network III: Genetic and hematological disorders, cell-to-cell signaling and interactions. These data offer many opportunities and new roadmaps for further functional studies to better understand the biology and pathogenic mechanisms of HS.