Linked Life Data

17401141

Source:http://linkedlifedata.com/resource/pubmed/id/17401141

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
21
pubmed:dateCreated
2007-5-21
pubmed:abstractText
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, "wrinkle-free" 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.
pubmed:grant
http://linkedlifedata.com/resource/pubmed/grant/C06RR015502, http://linkedlifedata.com/resource/pubmed/grant/K01DK075811, http://linkedlifedata.com/resource/pubmed/grant/P30 DK056341-06, http://linkedlifedata.com/resource/pubmed/grant/P30 DK056341-07, http://linkedlifedata.com/resource/pubmed/grant/P30DK052574, http://linkedlifedata.com/resource/pubmed/grant/P30DK056341, http://linkedlifedata.com/resource/pubmed/grant/P41RR000954, http://linkedlifedata.com/resource/pubmed/grant/P60DK020579, http://linkedlifedata.com/resource/pubmed/grant/R01 AR049269-01A1, http://linkedlifedata.com/resource/pubmed/grant/R01 AR049269-02, http://linkedlifedata.com/resource/pubmed/grant/R01 AR049269-03, http://linkedlifedata.com/resource/pubmed/grant/R01AR049269, http://linkedlifedata.com/resource/pubmed/grant/R01DK056260, http://linkedlifedata.com/resource/pubmed/grant/R37HL038180
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
May
pubmed:issn
0021-9258
pubmed:author
pubmed:issnType
Print
pubmed:day
25
pubmed:volume
282
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
15912-20
pubmed:dateRevised
2011-2-21
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
Keratinocyte-specific expression of fatty acid transport protein 4 rescues the wrinkle-free phenotype in Slc27a4/Fatp4 mutant mice.
pubmed:affiliation
Renal Division, Department of Internal Medicine, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
pubmed:publicationType
Journal Article, Research Support, N.I.H., Extramural