title: Cytoskeletal behaviour in spectrin and in band 3 deficient spherocytic red cells: evidence for differentiated splenic conditioning role.
abstract: Based on quantitative analysis of red cell membrane proteins, hereditary spherocytosis (HS) can be divided into two main groups including isolated or ankyrin combined spectrin deficiency and band 3 reduction. Protein methyl esterification catalysed by protein carboxyl methyl-transferase (PCMT type II; EC 2.1.1.77) is a post-biosynthetic modification which is involved in the metabolism of damaged membrane proteins. We utilized the evaluation of erythrocyte membrane protein methyl esterification as a marker of cytoskeletal disarray in seven HS subjects with spectrin reduction and in seven patients with HS due to band 3 deficiency. Our results support the notion that band 3 deficient erythrocytes are not affected by an extensive cytoskeletal derangement. On the contrary, we found a remarkable increase of membrane methylation in the unsplenectomized, spectrin-deficient. HS patients, suggesting a striking membrane skeleton disarray. This phenomenon was not observed in the spectrin-deficient red cells of splenectomized patients. Therefore in spectrin deficient erythrocytes the induction of cytoskeletal damage, specifically recognized by PCMT type II, could be one of the splenic steps producing conditioned spherocytes.
authors.affiliation: Institute of Biochemistry of Macromolecules, Second University of Naples, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Ingrosso
authors.forename: S
authors.initials: S
authors.lastname: D'Angelo
authors.forename: S
authors.initials: S
authors.lastname: Perrotta
authors.forename: G
authors.initials: G
authors.lastname: d'Urzo
authors.forename: A
authors.initials: A
authors.lastname: Iolascon
authors.forename: A F
authors.initials: AF
authors.lastname: Perna
authors.forename: P
authors.initials: P
authors.lastname: Galletti
authors.forename: V
authors.initials: V
authors.lastname: Zappia
authors.forename: E
authors.initials: E
authors.lastname: Miraglia del Giudice
mesh_terms: D000293:Adolescent; D000328:Adult; D001457:Anion Exchange Protein 1, Erythrocyte / Q000172:deficiency*; D002648:Child; D002675:Child, Preschool; D003599:Cytoskeleton / Q000648:ultrastructure*; D004912:Erythrocytes / Q000648:ultrastructure*; D005260:Female; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D008745:Methylation; D013049:Spectrin / Q000172:deficiency*; D013103:Spherocytosis, Hereditary / Q000378:metabolism / Q000473:pathology*; D013156:Splenectomy
chemical_list: D001457:Anion Exchange Protein 1, Erythrocyte; D008565:Membrane Proteins; D013049:Spectrin