A current review of fatty acid transport proteins (SLC27)

Stahl, Andreas

doi:10.1007/s00424-003-1106-z

Cited by 257 publications

(209 citation statements)

References 47 publications

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“…2g). Next, we examined mRNA expressions of FA transport proteins (FATP) 1, 3 and 4, which have a role in the uptake of long-chain FAs 20,21 . As shown in Fig.…”

Section: Blm Treatment Decreases Elovl6 Expression In the Lungmentioning

confidence: 99%

Deranged fatty acid composition causes pulmonary fibrosis in Elovl6-deficient mice

et al. 2013

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Despite the established role of alveolar type II epithelial cells for the maintenance of pulmonary function, little is known about the deregulation of lipid composition in the pathogenesis of pulmonary fibrosis. The elongation of long-chain fatty acids family member 6 (Elovl6) is a rate-limiting enzyme catalysing the elongation of saturated and monounsaturated fatty acids. Here we show that Elovl6 expression is significantly downregulated after an intratracheal instillation of bleomycin (BLM) and in human lung with idiopathic pulmonary fibrosis. Elovl6-deficient (Elovl6 À / À ) mice treated with BLM exhibit severe fibroproliferative response and derangement of fatty acid profile compared with wild-type mice. Furthermore, Elovl6 knockdown induces a change in fatty acid composition similar to that in Elovl6 À / À mice, resulting in induction of apoptosis, TGF-b1 expression and reactive oxygen species generation. Our findings demonstrate a previously unappreciated role for Elovl6 in the regulation of lung homeostasis, and in pathogenesis and exacerbation of BLM-induced pulmonary fibrosis.

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“…2g). Next, we examined mRNA expressions of FA transport proteins (FATP) 1, 3 and 4, which have a role in the uptake of long-chain FAs 20,21 . As shown in Fig.…”

Section: Blm Treatment Decreases Elovl6 Expression In the Lungmentioning

confidence: 99%

Deranged fatty acid composition causes pulmonary fibrosis in Elovl6-deficient mice

et al. 2013

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“…3 The identification of mammalian fatty acid transport proteins and associated acyl-CoA synthetases has strengthened the argument for protein-mediated transcellular fatty acid transport. 4 The data in ECs, however, remain scant. siRNA experiments in cell culture have indicated that fatty acid transport proteins 3 and 4 are required for VEGFB and 3-HIB (hydroxyisobutyrate)-induced endothelial fatty acid uptake, 1,2 but how these proteins function remains largely unknown.…”

Section: Article See P 1289mentioning

confidence: 99%

Does Endothelium Buffer Fat?

Ibrahim

Arany

2017

Circulation Research

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“…2 compose a family of transmembrane proteins that facilitate cellular long chain fatty acid uptake (1)(2)(3). The most widely expressed member of this family is FATP4 (4,5), which is encoded by Slc27a4 (solute carrier family 27 member 4 gene).…”

Section: Fatty Acid Transport Proteins (Fatps)mentioning

confidence: 99%

Keratinocyte-specific Expression of Fatty Acid Transport Protein 4 Rescues the Wrinkle-free Phenotype in Slc27a4/Fatp4 Mutant Mice

Moulson

Lin

White

et al. 2007

Journal of Biological Chemistry

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FATP4 (fatty acid transport protein 4; also known as SLC27A4) is the most widely expressed member of a family of six long chain fatty acid transporters. FATP4 is highly expressed in enterocytes and has therefore been proposed to be a major importer of dietary fatty acids. Two independent mutations in Fatp4 cause mice to be born with thick, tight, shiny, "wrinklefree" skin and a defective skin barrier; they die within hours of birth from dehydration and restricted movements. In contrast, induced keratinocyte-specific deficiency of FATP4 in adult mice causes only mild skin abnormalities. Therefore, whether the loss of FATP4 from skin or a systemic gestational metabolic defect causes the severe skin defects and neonatal lethality remain important unanswered questions. To investigate the basis for the phenotype, we first generated wild-type tetraploid/mutant diploid aggregates that should lead to rescue of any abnormalities caused by loss of FATP4 from the placenta. However, the skin phenotype was not ameliorated. We then generated transgenic mice expressing exogenous FATP4 either widely or specifically in suprabasal keratinocytes, and we bred the transgenes onto the Fatp4 ؊/؊ background. Both modes of FATP4 expression led to rescue of the neonatally lethal skin defects, and the resulting mice were viable and fertile. Keratinocyte expression of an FATP4 variant with mutations in the acyl-CoA synthetase domain did not provide any degree of rescue. We conclude that expression of FATP4 with an intact acyl-CoA synthetase domain in suprabasal keratinocytes is necessary for normal skin development and that FATP4 functions in establishing the cornified envelope. Fatty acid transport proteins (FATPs)2 compose a family of transmembrane proteins that facilitate cellular long chain fatty acid uptake (1-3). The most widely expressed member of this family is FATP4 (4, 5), which is encoded by Slc27a4 (solute carrier family 27 member 4 gene). The most robust expression of FATP4 is in intestinal epithelial cells (5), but its broad expression pattern is suggestive of functions in many organs.Surprisingly, the "wrinkle-free" phenotype we discovered in mice with a spontaneous mutation in Slc27a4 (Slc27a4 wrfr ) and the identical phenotype of mice with a targeted Slc27a4 mutation (Slc27a4) indicate critical roles for FATP4 in skin and hair development (6, 7). For these mutations, which both affect exon 3, homozygotes are born with tight, thick, shiny skin and a defective skin barrier; they die shortly after birth because of restricted movements and dehydration. These defects are reminiscent of human restrictive dermopathy, but this disease has recently been linked to mutations in zinc metalloproteinase STE24 (8 -10). Interestingly, deletion of Slc27a4 exons 2 and 3 (the first two coding exons; Slc27a4 tm1Asta ) results in embryonic lethality prior to embryonic day 9.5 (11). The reason for the striking difference in phenotypes remains unknown, but it may involve a partially functional truncated protein in the first two mutants or early ...

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A current review of fatty acid transport proteins (SLC27)

Cited by 257 publications

References 47 publications

Deranged fatty acid composition causes pulmonary fibrosis in Elovl6-deficient mice

Deranged fatty acid composition causes pulmonary fibrosis in Elovl6-deficient mice

Does Endothelium Buffer Fat?

Keratinocyte-specific Expression of Fatty Acid Transport Protein 4 Rescues the Wrinkle-free Phenotype in Slc27a4/Fatp4 Mutant Mice

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