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Titolo:
Diminished LDL receptor and high heparin binding of apolipoprotein E2 Sendai associated with lipoprotein glomenulopathy
Autore:
Hoffmann, MM; Scharnagl, H; Panagiotou, E; Banghard, WT; Wieland, H; Marz, W;
Indirizzi:
Univ Freiburg, Div Clin Chem, Med Clin, Dept Med, D-79106 Freiburg, Germany Univ Freiburg Freiburg Germany D-79106 pt Med, D-79106 Freiburg, Germany
Titolo Testata:
JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY
fascicolo: 3, volume: 12, anno: 2001,
pagine: 524 - 530
SICI:
1046-6673(200103)12:3<524:DLRAHH>2.0.ZU;2-F
Fonte:
ISI
Lingua:
ENG
Soggetto:
LOW-DENSITY-LIPOPROTEIN; III HYPERLIPOPROTEINEMIA; HUMAN-FIBROBLASTS; E POLYMORPHISM; ACID DELETION; E ISOFORMS; GLOMERULOPATHY; MUTATION; VARIANT; PROTEIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
44
Recensione:
Indirizzi per estratti:
Indirizzo: Hoffmann, MM Univ Freiburg, Div Clin Chem, Med Clin, Dept Med, Hugstetter Str 55, D-79106 Freiburg, Germany Univ Freiburg Hugstetter Str 55 FreiburgGermany D-79106 any
Citazione:
M.M. Hoffmann et al., "Diminished LDL receptor and high heparin binding of apolipoprotein E2 Sendai associated with lipoprotein glomenulopathy", J AM S NEPH, 12(3), 2001, pp. 524-530

Abstract

Variants of apolipoprotein E (apoE) have been linked to lipoprotein glomerulopathy, a new glomerular disease characterized by the deposition of lipoproteins in mesangial capillaries. One third of affected patients are heterozygous for apoE2 Sendai (Arg(145)->Pro). Variants of apoE can also produce type III hyperlipoproteinemia (HLP). Recessive type III HLP is caused by apoE2 (Arg(158)->Cys), a mutant with diminished low-density lipoprotein (LDL)receptor binding but half-normal heparin binding. Dominant type III HLP iscaused by mutations that markedly alter heparin binding but modestly reduce receptor binding. This study examined whether apoE2 Sendai (Arg(145)->Pro) was functionally different from type In HLP-producing apoE variants by expressing apoE3, apoE2 (Arg(158)->Cys), apoE1 (Arg(146)->Glu), a dominant apoE variant, and apoE2 Sendai (Arg(145)->Pro) in the baculovirus system. LDLreceptor binding was studied using recombinant apoE complexed to phospholipid vesicles and to very low-density lipoprotein from a patient with familiar apoE deficiency. Compared with apoE3, receptor-binding activities of apoE2 (Arg(158)->Cys), apoE1 (Arg(146)->Glu), and apoE2 Sendal (Arg(145)->Pro)all were less than 5%. Heparin-binding activities were 53%, 23%, and 66%, respectively, of apoE3. The distribution of apoE2 Sendai among the major plasma lipoprotein fractions was similar to that of apoE3 and apoE2 (Arg(158)->Cys). ApoE2 Sendai (Arg(145)->Pro) represents the only known mutation within the heparin-binding domain of apoE (residues 142 through 147), revealing diminished receptor binding and almost normal heparin binding. These unique characteristics of apoE2 Sendai (Arg(145)->Pro) may relate to the development of lipoprotein glomerulopathy.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/09/20 alle ore 19:41:16