+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Mutant oocytic low density lipoprotein receptor gene family member causes atherosclerosis and female sterility



Mutant oocytic low density lipoprotein receptor gene family member causes atherosclerosis and female sterility



Proceedings of the National Academy of Sciences of the United States of America 92(21): 9905-9909



The so-called very low density lipoprotein receptors (VLDLRs) are related to the LDLR gene family. So far, naturally occurring mutations have only been described for the prototype LDLR; in humans, they cause familial hypercholesterolemia. Here we describe a naturally occurring mutation in a VLDLR that causes a dramatic abnormal phenotype. Hens of the mutant restricted-ovulator chicken strain carry a single mutation, lack functional oocyte receptors, are sterile, and display severe hyperlipidemia with associated premature atherosclerosis. The mutation converts a cysteine residue into a serine, resulting in an unpaired cysteine and greatly reduced expression of the mutant avian VLDLR on the oocyte surface. Extraoocytic cells in the mutant produce higher than normal amounts of a differentially spliced form of the receptor that is characteristic for somatic cells but absent from germ cells.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 009066412

Download citation: RISBibTeXText

PMID: 7568242

DOI: 10.2307/2368585


Related references

Isolation and characterization of LRP6, a novel member of the low density lipoprotein receptor gene family. Biochemical and Biophysical Research Communications 248(3): 879-888, 1998

Molecular characterization and expression of the low-density lipoprotein receptor-related protein-10, a new member of the LDLR gene family. Biochemical and Biophysical Research Communications 391(1): 1110-1115, 2010

Complete cloning and sequencing of rat gp330/megalin, a distinctive member of the low density lipoprotein receptor gene family. Proceedings of the National Academy of Sciences of the United States of America 91(21): 9725-9729, 1994

Glycoprotein 330, a member of the low density lipoprotein receptor family, binds lipoprotein lipase in vitro. Journal of Biological Chemistry 268(19): 14176-14181, 1993

The somatic cell-specific low density lipoprotein receptor-related protein of the chicken. Close kinship to mammalian low density lipoprotein receptor gene family members. Journal of Biological Chemistry 269(1): 212-219, 1994

Germ cell-somatic cell dichotomy of a low-density lipoprotein receptor gene family member in testis. Dna and Cell Biology 16(1): 35-43, 1997

Novel synaptic protein, short form ldl receptor related protein , a member of low density lipoprotein gene family in the rat brain. Society for Neuroscience Abstract Viewer & Itinerary Planner : Abstract No 746 4, 2002

Defects in the low density lipoprotein receptor gene affect lipoprotein (a) levels: multiplicative interaction of two gene loci associated with premature atherosclerosis. Proceedings of the National Academy of Sciences of the United States of America 86(11): 4171-4174, 1989

Hypercholesterolemia and atherosclerosis in low density lipoprotein receptor mutant rats. Biochemical and Biophysical Research Communications 418(3): 553-558, 2012

Cloning of a novel member of the low-density lipoprotein receptor family. Gene (amsterdam). 216(1): 103-111,. 17, 1998

Inhibition by a coantioxidant of aortic lipoprotein lipid peroxidation and atherosclerosis in apolipoprotein E and low density lipoprotein receptor gene double knockout mice. Faseb Journal 13(6): 667-675, 1999

The putative tumor suppressor LRP1B, a novel member of the low density lipoprotein (LDL) receptor family, exhibits both overlapping and distinct properties with the LDL receptor-related protein. Journal of Biological Chemistry 276(31): 28889-28896, 2001

Elements of neural adhesion molecules and a yeast vacuolar protein sorting receptor are present in a novel mammalian low density lipoprotein receptor family member. Journal of Biological Chemistry 271(40): 24761-8, 1996

Increased low-density lipoprotein oxidation and impaired high-density lipoprotein antioxidant defense are associated with increased macrophage homing and atherosclerosis in dyslipidemic obese mice: LCAT gene transfer decreases atherosclerosis. Circulation 107(12): 1640-1646, 2003

Tesaglitazar, a dual peroxisome proliferator-activated receptor alpha/gamma agonist, reduces atherosclerosis in female low density lipoprotein receptor deficient mice. Atherosclerosis 195(1): 100-109, 2007