Decreased expression of Intestinal I- and L-FABP levels in rare human genetic lipid malabsorption syndromes.
Histochem Cell Biol
; 128(2): 115-23, 2007 Aug.
Article
em En
| MEDLINE
| ID: mdl-17605029
ABSTRACT
We investigated, for the first time, the expression of I- and L-FABP in two very rare hereditary lipid malabsorption syndromes as compared with normal subjects. Abetalipoproteinemia (ABL) and Anderson's disease (AD) are characterized by an inability to export alimentary lipids as chylomicrons that result in fat loading of enterocytes. Duodeno-jejunal biopsies were obtained from 14 fasted normal subjects, and from four patients with ABL and from six with AD. Intestinal FABP expression was investigated by immuno-histochemistry, western blot, ELISA and Northern blot analysis. In contrast to normal subjects, the cellular immunostaining for both FABPs was clearly decreased in patients, as the enterocytes became fat-laden. In patients with ABL, the intestinal contents of I- (60.7 +/- 13.38 ng/mg protein) and L-FABP (750.3 +/- 121.3 ng/mg protein) are significantly reduced (50 and 35%, P < 0.05, respectively) as compared to normal subjects (I-135.3 +/- 11.1 ng, L-1211 +/- 110 ng/mg protein). In AD, the patients also exhibited decreased expression (50%, P < 0.05; I-59 +/- 11.88 ng, L-618.2 +/- 104.6 ng/mg protein). Decreased FABP expression was not associated with decreased mRNA levels. The results suggest that enterocytes might regulate intracellular FABP content in response to intracellular fatty acids, which we speculate may act as lipid sensors to prevent their intracellular transport.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Abetalipoproteinemia
/
Proteínas de Ligação a Ácido Graxo
/
Mucosa Intestinal
/
Erros Inatos do Metabolismo Lipídico
/
Síndromes de Malabsorção
Limite:
Adolescent
/
Adult
/
Child
/
Female
/
Humans
/
Male
Idioma:
En
Revista:
Histochem Cell Biol
Assunto da revista:
CITOLOGIA
/
HISTOCITOQUIMICA
Ano de publicação:
2007
Tipo de documento:
Article
País de afiliação:
França