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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
 

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LDLR  -  low density lipoprotein receptor

Macaca mulatta

 
 
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Disease relevance of LDLR

 

High impact information on LDLR

  • Moreover, from the breeding of one of the affected male offspring with six unrelated normocholesterolemic female monkeys, eight offspring were generated of which three were hypercholesterolemic on a cholesterol-free diet and exhibited the same degree of LDL-R deficiency as shown by studies in skin fibroblast cultures [2].
  • We previously described a family of rhesus monkeys in which three out of six members had a spontaneous hypercholesterolemia related to a decrease in number of low density lipoprotein receptors (LDL-R) (Scanu et al. 1988. J. Lipid Res. 29: 1671-1681) [2].
  • Hepatic LDL receptor and HMG-CoA reductase activities were not detectable in the high-responders, while the low-responders expressed a reduced number of LDL receptors of normal affinity [3].
  • Taken together our results demonstrate a successful transmission to second generation animals of the LDL-R deficiency phenotype and provide evidence that this phenotype correlates well with plasma LDL levels but not Lp[a] [2].
  • As a result of a lack of a functional LDL receptor pathway, pigeon smooth muscle cells do not maintain cholesterol homeostasis through the controlled uptake of exogenous LDL cholesterol, as do mammalian cells [4].
 

Chemical compound and disease context of LDLR

  • We conclude that a genetically determined LDL receptor deficiency was responsible, in part, for the spontaneous hypercholesterolemia observed in three out of the six family members and that this deficiency accounted for the hyperresponsiveness to a dietary fat and cholesterol challenge by the dam and the two offspring, B-1000 and B-7643 [5].
 

Biological context of LDLR

  • The action of the novel antihypertensive calcium antagonist monatepil on the hepatic LDL receptor was investigated at the gene expression level to clarify the mechanism of its hypolipidemic effect [6].
 

Anatomical context of LDLR

 

Associations of LDLR with chemical compounds

 

Analytical, diagnostic and therapeutic context of LDLR

  • In conclusion, HD-Ad-mediated LDLR gene therapy is effective in conferring long-term protection against atherosclerosis in a mouse model of familial hypercholesterolemia [9].

References

  1. Low density lipoprotein receptor activity on skin fibroblasts from rhesus monkeys with diet-induced or spontaneous hypercholesterolemia. Guertler, L.S., St Clair, R.W. J. Biol. Chem. (1980) [Pubmed]
  2. Rhesus monkey model of familial hypercholesterolemia: relation between plasma Lp[a] levels, apo[a] isoforms, and LDL-receptor function. Neven, L., Khalil, A., Pfaffinger, D., Fless, G.M., Jackson, E., Scanu, A.M. J. Lipid Res. (1990) [Pubmed]
  3. Hepatic low density lipoprotein receptors, HMG-CoA reductase, and plasma lipids and apolipoproteins in high- and low-responding rhesus monkeys: effect of cholestyramine treatment. Dory, L., Bhattacharyya, A., Strong, J., Chappuis, C. J. Lipid Res. (1990) [Pubmed]
  4. Cholesterol metabolism in pigeon aortic smooth muscle cells lacking a functional low density lipoprotein receptor pathway. Randolph, R.K., Smith, B.P., St Clair, R.W. J. Lipid Res. (1984) [Pubmed]
  5. Genetically determined hypercholesterolemia in a rhesus monkey family due to a deficiency of the LDL receptor. Scanu, A.M., Khalil, A., Neven, L., Tidore, M., Dawson, G., Pfaffinger, D., Jackson, E., Carey, K.D., McGill, H.C., Fless, G.M. J. Lipid Res. (1988) [Pubmed]
  6. Up-regulation of hepatic LDL receptor gene expression by monatepil, a novel calcium antagonist, in high cholesterol diet-fed Japanese monkeys. Notake, M., Kondo, Y., Nomura, H., Nakano, K., Hosoki, K., Miyazaki, M. Am. J. Hypertens. (1994) [Pubmed]
  7. Pigeon aortic smooth muscle cells lack a functional low density lipoprotein receptor pathway. Randolph, R.K., St Clair, R.W. J. Lipid Res. (1984) [Pubmed]
  8. Squalene synthase inhibitors reduce plasma triglyceride through a low-density lipoprotein receptor-independent mechanism. Hiyoshi, H., Yanagimachi, M., Ito, M., Saeki, T., Yoshida, I., Okada, T., Ikuta, H., Shinmyo, D., Tanaka, K., Kurusu, N., Tanaka, H. Eur. J. Pharmacol. (2001) [Pubmed]
  9. Low-density lipoprotein receptor gene therapy using helper-dependent adenovirus produces long-term protection against atherosclerosis in a mouse model of familial hypercholesterolemia. Nomura, S., Merched, A., Nour, E., Dieker, C., Oka, K., Chan, L. Gene Ther. (2004) [Pubmed]
 
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