title: Lipid metabolism in phosphatidylinositol transfer protein alpha-deficient vibrator mice.
abstract: Mice that are homozygous for the vibrator mutation express 65-85% less phosphatidylinositol transfer protein alpha (PITPalpha) than their wild type litter mates. By postnatal day 10-12 (P10-12) they exhibit signs of neurodegeneration and die prematurely by P40. In the present study, we examine the lipid content of brain, liver, and mammary glands from these animals. Lipid-mediated signal transduction is evaluated in primary fibroblast cultures. With respect to the lipid make-up of brain and liver, we report that there is a significant increase (2- to 4-fold) in the neutral lipids present in the livers of vb/vb animals when compared with wild type (+/+) litter mates. No significant changes are observed in the brains of these animals. The mammary glands of vb/vb mice are underdeveloped with respect to ductal and alveolar structures, and the fat pad is composed of predominantly brown adipose tissue rather than the white adipose tissue characteristic of age-matched wild type litter mates. No differences are observed in any aspect of lipid-mediated signal transduction.
authors.affiliation: Department of Urology, NYU School of Medicine, New York, NY 10016, USA. mem6@nyu.edu
authors.forename: Marie E
authors.initials: ME
authors.lastname: Monaco
authors.forename: James
authors.initials: J
authors.lastname: Kim
authors.forename: WeiFeng
authors.initials: W
authors.lastname: Ruan
authors.forename: Rosemary
authors.initials: R
authors.lastname: Wieczorek
authors.forename: David L
authors.initials: DL
authors.lastname: Kleinberg
authors.forename: Paul D
authors.initials: PD
authors.lastname: Walden
mesh_terms: D000273:Adipose Tissue / Q000378:metabolism*; D000818:Animals; D001921:Brain / Q000378:metabolism*; D002352:Carrier Proteins; D002478:Cells, Cultured; D005347:Fibroblasts / Q000378:metabolism; D050356:Lipid Metabolism; D008099:Liver / Q000378:metabolism*; D008321:Mammary Glands, Animal / Q000378:metabolism*; D008565:Membrane Proteins / Q000172:deficiency*; D051379:Mice; D008810:Mice, Inbred C57BL; D010713:Phosphatidylcholines / Q000378:metabolism*; D010716:Phosphatidylinositols / Q000378:metabolism*; D049294:Phospholipid Transfer Proteins; D015398:Signal Transduction / Q000187:drug effects / Q000502:physiology; D014018:Tissue Distribution; D014667:Vasopressins / Q000494:pharmacology
chemical_list: D002352:Carrier Proteins; D008565:Membrane Proteins; D010713:Phosphatidylcholines; D010716:Phosphatidylinositols; D049294:Phospholipid Transfer Proteins; D014667:Vasopressins