Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
2008-3-4
pubmed:abstractText
The cellular uptake of vitamin A from its RBP4-bound circulating form (holo-RBP4) is a homeostatic process that evidently depends on the multidomain membrane protein STRA6. In humans, mutations in STRA6 are associated with Matthew-Wood syndrome, manifested by multisystem developmental malformations. Here we addressed the metabolic basis of this inherited disease. STRA6-dependent transfer of retinol from RBP4 into cultured NIH 3T3 fibroblasts was enhanced by lecithin:retinol acyltransferase (LRAT). The retinol transfer was bidirectional, strongly suggesting that STRA6 acts as a retinol channel/transporter. Loss-of-function analysis in zebrafish embryos revealed that Stra6 deficiency caused vitamin A deprivation of the developing eyes. We provide evidence that, in the absence of Stra6, holo-Rbp4 provokes nonspecific vitamin A excess in several embryonic tissues, impairing retinoic acid receptor signaling and gene regulation. These fatal consequences of Stra6 deficiency, including craniofacial and cardiac defects and microphthalmia, were largely alleviated by reducing embryonic Rbp4 levels by morpholino oligonucleotide or pharmacological treatments.
pubmed:grant
pubmed:commentsCorrections
http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-10192400, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-10232633, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-10469643, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-11017081, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-11157778, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-11688558, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-12050134, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-12167406, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-12668631, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-14585362, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-14684738, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-14704333, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-15653502, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-15654843, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-15680360, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-15911617, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16034410, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16316642, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16402912, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16537499, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16611695, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16756510, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-16929533, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-17098734, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-17255476, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-17273977, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-17339024, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-17503335, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-2218545, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-2825025, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-2825036, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-7940754, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8041782, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8354401, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8464695, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8565830, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8565839, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8589427, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8801176, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-8954734, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-9203140, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-945163, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-9758748, http://linkedlifedata.com/resource/pubmed/commentcorrection/18316031-9831649
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
http://linkedlifedata.com/resource/pubmed/chemical/Acyltransferases, http://linkedlifedata.com/resource/pubmed/chemical/Membrane Proteins, http://linkedlifedata.com/resource/pubmed/chemical/Morpholines, http://linkedlifedata.com/resource/pubmed/chemical/Oligonucleotides, Antisense, http://linkedlifedata.com/resource/pubmed/chemical/RBP4 protein, human, http://linkedlifedata.com/resource/pubmed/chemical/Rbp4 protein, zebrafish, http://linkedlifedata.com/resource/pubmed/chemical/Retinol-Binding Proteins, Plasma, http://linkedlifedata.com/resource/pubmed/chemical/STRA6 protein, human, http://linkedlifedata.com/resource/pubmed/chemical/Tretinoin, http://linkedlifedata.com/resource/pubmed/chemical/Vitamin A, http://linkedlifedata.com/resource/pubmed/chemical/Zebrafish Proteins, http://linkedlifedata.com/resource/pubmed/chemical/lecithin-retinol acyltransferase
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
1550-4131
pubmed:author
pubmed:issnType
Print
pubmed:volume
7
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
258-68
pubmed:dateRevised
2010-12-3
pubmed:meshHeading
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