Herrmann, Thomas and van der Hoeven, Frank and Groene, Hermann-Josef and Stewart, Adrian Francis and Langbein, Lutz and Kaiser, Iris and Liebisch, Gerhard and Gosch, Isabella and Buchkremer, Florian and Drobnik, Wolfgang and Schmitz, Gerd and Stremmel, Wolfgang (2003) Mice with targeted disruption of the fatty acid transport protein 4 (Fatp 4, SlC27a4) gene show features of lethal restrictive dermopathy. JOURNAL OF CELL BIOLOGY, 161 (6). pp. 1105-1115. ISSN 0021-9525
Full text not available from this repository.Abstract
The fatty acid transport protein family is a group of evolutionarily conserved proteins that are involved in the cellular uptake and metabolism of long and very long chain fatty acids. However, little is known about their respective physiological roles. To analyze the functional significance of fatty acid transport protein 4 (Fatp4, Slc27a4), we generated mice with a targeted disruption of the Fatp4 gene. Fatp4-null mice displayed features of a neonatally lethal restrictive dermopathy. Their skin was characterized by hyperproliferative hyperkeratosis with a disturbed epidermal barrier, a flat dermal-epidermal junction, a reduced number of pilo-sebaceous structures, and a compact dermis. The rigid skin consistency resulted in an altered body shape with facial dysmorphia, generalized joint flexion contractures, and impaired movement including suckling and breathing deficiencies. Lipid analysis demonstrated a disturbed fatty acid composition of epidermal ceramides, in particular a decrease in the C26:0 and C26: 0-OH fatty acid substitutes. These findings reveal a previously unknown, essential function of Fatp4 in the formation of the epidermal barrier.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ACYL-COA SYNTHETASE; TANDEM MASS-SPECTROMETRY; BARRIER FUNCTION; PERMEABILITY BARRIER; TIGHT JUNCTIONS; STRATUM-CORNEUM; SKIN; EXPRESSION; CLONING; COMPONENTS; ceramides; epidermis; Fatp4; fatty acid metabolism; fatty acid transport |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 11 Aug 2021 04:44 |
| Last Modified: | 11 Aug 2021 04:44 |
| URI: | https://pred.uni-regensburg.de/id/eprint/38892 |
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