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Titolo:
Increased erythrocyte content of Ca2+ in patients with Tarui's disease
Autore:
Waldenstrom, A; Engstrom, I; Ronquist, G;
Indirizzi:
Univ Uppsala Hosp, Dept Clin Chem, S-75185 Uppsala, Sweden Univ Uppsala Hosp Uppsala Sweden S-75185 n Chem, S-75185 Uppsala, Sweden Norrlands Univ Hosp, Dept Internal Med, S-90185 Umea, Sweden Norrlands Univ Hosp Umea Sweden S-90185 ternal Med, S-90185 Umea, Sweden
Titolo Testata:
JOURNAL OF INTERNAL MEDICINE
fascicolo: 1, volume: 249, anno: 2001,
pagine: 97 - 102
SICI:
0954-6820(200101)249:1<97:IECOCI>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
INHERITED PHOSPHOFRUCTOKINASE DEFICIENCY;
Keywords:
calcium ions; erythrocyte; human; permeability; Tarui's disease;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
13
Recensione:
Indirizzi per estratti:
Indirizzo: Ronquist, G Univ Uppsala Hosp, Dept Clin Chem, S-75185 Uppsala, Sweden Univ Uppsala Hosp Uppsala Sweden S-75185 185 Uppsala, Sweden
Citazione:
A. Waldenstrom et al., "Increased erythrocyte content of Ca2+ in patients with Tarui's disease", J INTERN M, 249(1), 2001, pp. 97-102

Abstract

Objective. To establish by flow cytometry and fluorophores an increased calcium ion load in erythrocytes of four patients with Tarui's disease. Design. Calcium ion levels were determined in erythrocytes of patients andcontrols under normal and energy-deprived conditions. Adenylates were measured to assess energy status of incubated erythrocytes. Setting. The experiments were carried out at the Department of Clinical Chemistry of the University Hospital of Uppsala, Sweden. Subjects. Four family members with Tarui's disease participated in the study. The proband (patient 1) was a 39- year-old male; patients (male, aged 46 years) 2 and 3 (female, 30 years) were his two siblings. Patient 4 (male,16 years) was the son of patient 2. Intervention. None. Main outcome measures. Calcium ion homeostasis was measured under basic conditions and under energy-deprived conditions and related to cellular adenylate content. Results. All patients showed enhanced erythrocyte calcium ion loading compared to controls under energy-deprived conditions. Under normal conditions,however, three out of four patients showed an increased erythrocyte calcium ion level compared to controls. Conclusions. We conclude that erythrocytes from patients with Tarui's disease have an increased Ca2+ permeability, initiating compensatory mechanismsinvolving increased Ca2+ pump activity and increased glycolytic flux, which are not always sufficient to keep erythrocyte calcium ion concentration within physiological range.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 16:52:24