Decreased expression of Intestinal I- and L-FABP levels in rare human genetic lipid malabsorption syndromes

Autor: André Bado, Claude Besmond, Sandra Guilmeau, J. P. Laigneau, V. Petit, H. Devaud, L. Ferkdadji, M. E. Samson-Bouma, R. Bouvier, L. P. Aggerbeck, N. Brousse, Isabelle Niot
Přispěvatelé: Centre de recherche biomédicale Bichat-Beaujon ( CRB3 ), Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre des Sciences du Goût ( CSG ), Institut National de la Recherche Agronomique ( INRA ) -Université de Bourgogne ( UB ) -Centre National de la Recherche Scientifique ( CNRS ), Laboratoire de Physiologie de la Nutrition, Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon ( ENSBANA ), Service d'anatomie pathologique [CHU Necker], CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP), Laboratoire Central d'Anatomie et de Cytologie, Hôpital Herriot, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Service d'anatomie et de cytologie pathologique, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Handicaps génétiques de l'enfant ( Inserm U393 ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Paris Descartes - Paris 5 ( UPD5 ), Centre de génétique moléculaire ( CGM ), Centre National de la Recherche Scientifique ( CNRS ), Centre de recherche biomédicale Bichat-Beaujon (CRB3), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre des Sciences du Goût (CSG), Institut National de la Recherche Agronomique (INRA)-Université de Bourgogne (UB)-Centre National de la Recherche Scientifique (CNRS), Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Handicaps génétiques de l'enfant (Inserm U393), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de génétique moléculaire (CGM), Centre National de la Recherche Scientifique (CNRS), Etablissement National d'Enseignement Supérieur Agronomique de Dijon (ENESAD)-Institut National de la Recherche Agronomique (INRA)-Université de Bourgogne (UB)-Centre National de la Recherche Scientifique (CNRS), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Université Paris Diderot - Paris 7 (UPD7)-Hôpital Robert Debré-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)
Rok vydání: 2007
Předmět:
Adult
Male
medicine.medical_specialty
Histology
Adolescent
Biology
Fatty Acid-Binding Proteins
Fatty acid-binding protein
Lipid Metabolism
Inborn Errors
03 medical and health sciences
0302 clinical medicine
Intestinal mucosa
Western blot
Malabsorption Syndromes
Internal medicine
medicine
Humans
[SDV.BBM]Life Sciences [q-bio]/Biochemistry
Molecular Biology
Northern blot
RNA
Messenger
Intestinal Mucosa
Child
Molecular Biology
[ SDV.BBM ] Life Sciences [q-bio]/Biochemistry
Molecular Biology
030304 developmental biology
0303 health sciences
medicine.diagnostic_test
Abetalipoproteinemia
Cell Biology
medicine.disease
Immunohistochemistry
Medical Laboratory Technology
Endocrinology
030220 oncology & carcinogenesis
lipids (amino acids
peptides
and proteins)
Female
Intracellular
Immunostaining
Chylomicron
Zdroj: Histochemie / Histochemistry Histochimie
Histochemie / Histochemistry Histochimie, 2007, 128 (2), pp.115-23. 〈10.1007/s00418-007-0302-x〉
Histochemie / Histochemistry Histochimie, 2007, 128 (2), pp.115-23. ⟨10.1007/s00418-007-0302-x⟩
ISSN: 0948-6143
Popis: 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.
Databáze: OpenAIRE
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