低密度脂蛋白受体
低密度脂蛋白受体（Low-Density Lipoprotein (LDL) Receptor），又称LDL受体，为一种膜镶嵌式蛋白质，全长有839个氨基酸（扣掉21氨基酸长的讯息肽）[1]。本受体调控低密度脂蛋白（LDL）的胞吞作用。此种受体专门辨认LDL颗粒上的B100脂蛋白，也可辨认乳糜微粒残体及中间密度脂蛋白（IDL，极低密度脂蛋白的残体）上的Apo E。人体LDL受体的基因编码为LDLR基因[2][3]。属于低密度脂蛋白受体家族[4]。
Michael S. Brown和Joseph L. Goldstein即因发现LDL受体，以及其与胆固醇代谢的关系[5]，而获得1985年诺贝尔生理医学奖[6]。
临床价值
功能
LDL-胆固醇会和衔接蛋白（adaptin）结合，并在细胞膜中格形蛋白（clathrin）围住的孔洞中，与LDL受体结合成“LDL受体复合物”。此时，格形蛋白会将LDL-胆固醇吞噬入细胞内，形成囊泡。
突变
参考文献
- ^ Südhof TC, Goldstein JL, Brown MS, Russell DW. The LDL receptor gene: a mosaic of exons shared with different proteins. Science. May 1985, 228 (4701): 815–22. PMID 2988123. doi:10.1126/science.2988123.
- ^ Francke U, Brown MS, Goldstein JL. Assignment of the human gene for the low density lipoprotein receptor to chromosome 19: synteny of a receptor, a ligand, and a genetic disease. Proc. Natl. Acad. Sci. U.S.A. May 1984, 81 (9): 2826–30. PMC 345163
. PMID 6326146. doi:10.1073/pnas.81.9.2826.
- ^ Lindgren V, Luskey KL, Russell DW, Francke U. Human genes involved in cholesterol metabolism: chromosomal mapping of the loci for the low density lipoprotein receptor and 3-hydroxy-3-methylglutaryl-coenzyme A reductase with cDNA probes. Proc. Natl. Acad. Sci. U.S.A. December 1985, 82 (24): 8567–71. PMC 390958 . PMID 3866240. doi:10.1073/pnas.82.24.8567.
- ^ Nykjaer A, Willnow TE. The low-density lipoprotein receptor gene family: a cellular Swiss army knife?. Trends Cell Biol. June 2002, 12 (6): 273–80 [2015-01-26]. PMID 12074887. doi:10.1016/S0962-8924(02)02282-1. （原始内容存档于2018-05-01）.
- ^ Brown MS, Goldstein JL. How LDL Receptors Influence Cholesterol and Atherosclerosis. Scientific American. 1984, 251 (3): 52–60. PMID 6390676. doi:10.1038/scientificamerican0984-52.
- ^ The Nobel Prize in Physiology or Medicine 1985 (新闻稿). The Royal Swedish Academy of Science. 1985 [2010-07-01]. （原始内容存档于2011-01-02）.
- ^ Low Density Lipoprotein Receptor. LOVD v.1.1.0 - Leiden Open Variation Database. [2015-01-26]. （原始内容存档于2016-01-28）.
延伸阅读
- Brown MS, Goldstein JL. Receptor-mediated endocytosis: insights from the lipoprotein receptor system.. Proc. Natl. Acad. Sci. U.S.A. 1979, 76 (7): 3330–7. PMC 383819 . PMID 226968. doi:10.1073/pnas.76.7.3330.
- Hobbs HH, Brown MS, Goldstein JL. Molecular genetics of the LDL receptor gene in familial hypercholesterolemia.. Hum. Mutat. 1993, 1 (6): 445–66. PMID 1301956. doi:10.1002/humu.1380010602.
- Fogelman AM, Van Lenten BJ, Warden C; et al. Macrophage lipoprotein receptors.. J. Cell Sci. Suppl. 1989, 9: 135–49. PMID 2855802.
- Barrett PH, Watts GF. Shifting the LDL-receptor paradigm in familial hypercholesterolemia: novel insights from recent kinetic studies of apolipoprotein B-100 metabolism.. Atherosclerosis. Supplements. 2002, 2 (3): 1–4. PMID 11923121. doi:10.1016/S1567-5688(01)00012-5.
- May P, Bock HH, Herz J. Integration of endocytosis and signal transduction by lipoprotein receptors.. Sci. STKE. 2003, 2003 (176): PE12. PMID 12671190. doi:10.1126/stke.2003.176.pe12.
- Gent J, Braakman I. Low-density lipoprotein receptor structure and folding.. Cell. Mol. Life Sci. 2004, 61 (19-20): 2461–70. PMID 15526154. doi:10.1007/s00018-004-4090-3.
外部链接
- Description of LDL receptor pathway at the Brown - Goldstein Laboratory webpage（页面存档备份，存于互联网档案馆）
- 医学主题词表（MeSH）：LDL+Receptor
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