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Titolo:
Lectin analyses of glycoprotein hormones in patients with congenital disorders of glycosylation
Autore:
Ferrari, MC; Parini, R; Di Rocco, M; Radetti, G; Beck-Peccoz, P; Persani, L;
Indirizzi:
Univ Milan, Ist Auxol Italiano IRCCS, Lab Ric Endocrinol, Inst Endocrine Sci, I-20145 Milan, Italy Univ Milan Milan Italy I-20145 Inst Endocrine Sci, I-20145 Milan, Italy Ist Clin Perfezionamento, Clin Pediat De Marchi, Milan, Italy Ist Clin Perfezionamento Milan Italy lin Pediat De Marchi, Milan, Italy Ist Giannina Gaslini, Div Pediat 2, I-16148 Genoa, Italy Ist Giannina Gaslini Genoa Italy I-16148 Pediat 2, I-16148 Genoa, Italy Osped Reg Bolzano, Reparto Pediat, Bolzano, Italy Osped Reg Bolzano Bolzano Italy Bolzano, Reparto Pediat, Bolzano, Italy
Titolo Testata:
EUROPEAN JOURNAL OF ENDOCRINOLOGY
fascicolo: 4, volume: 144, anno: 2001,
pagine: 409 - 416
SICI:
0804-4643(200104)144:4<409:LAOGHI>2.0.ZU;2-3
Fonte:
ISI
Lingua:
ENG
Soggetto:
SYNDROME TYPE-I; PHOSPHOMANNOSE ISOMERASE DEFICIENCY; ASPARAGINE-LINKED OLIGOSACCHARIDES; THYROXINE-BINDING GLOBULIN; CDG SYNDROME; CIRCULATING THYROTROPIN; TRANSFERRIN; MUTATIONS; BIOACTIVITY; ISOFORMS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
39
Recensione:
Indirizzi per estratti:
Indirizzo: Persani, L Univ Milan, Ist Auxol Italiano IRCCS, Lab Ric Endocrinol, Inst Endocrine Sci, Via Ariosto 13, I-20145 Milan, Italy Univ Milan Via Ariosto 13 Milan Italy I-20145 145 Milan, Italy
Citazione:
M.C. Ferrari et al., "Lectin analyses of glycoprotein hormones in patients with congenital disorders of glycosylation", EUR J ENDOC, 144(4), 2001, pp. 409-416

Abstract

Objective: The congenital disorders of glycosylation (CDGs) are progressive multisystemic disorders characterized by a heterogeneous deficiency of the carbohydrate moieties in various structural and circulating glycoproteins, representing a natural model for glycoprotein hormone studies. Here, we studied the carbohydrate moiety of circulating glycoprotein hormones in fourpatients with a clinical suspicion of CDGs,Methods: The diagnosis of CDG-I was confirmed in two out of the four casesby transferrin isoelectrofocusing (IEF) and/or carbohydrate-deficient transferrin (CDT) test. The carbohydrate moiety of serum endocrine-related glycoproteins was investigated by means of Ricin (immunopurified thyrotropin (TSH)) and Concanavalin A (Con-A) (TSH, follicle-stimulating horomone, alpha-subunit and thyroglobulin) lectin affinity chromatography measurement. Results: CDT concentrations were very high in the two patients with CDG-I and moderately enhanced in the remaining two. In the two CDG-I patients, Ricin analysis of immunopurified TSH showed a severe impairment of Lectin binding, both before and after neuroaminidase treatment, indicating a nearly complete lack of terminal sialic acid and galactose residues. In these two cases, Con-A analysis showed a significant prevalence of firmly bound isoforms with poorly processed carbohydrate chains. In the remaining two cases with unknown CDG classification, TSH binding pattern to Ricin was modestly affected and Con-A analysis showed the prevalence of weakly bound glycoprotein isoforms. Conclusions: The results of Ricin analyses in all four patients were consistent with the CDT test and/or serum transferrin IEE The severe alteration of TSH binding pattern to Ricin seems to be characteristic of CDG-I. Nevertheless, TSH biological properties are not severely altered, as normal thyroid function was found in both cases.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/09/20 alle ore 09:29:43