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 |
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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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