cd00112

LDLa

CDD entry
Member databaseCDD
CDD typedomain
Short nameLDLa
SetLDLa

Description

Low Density Lipoprotein Receptor Class A domain, a cysteine-rich repeat that plays a central role in mammalian cholesterol metabolism; the receptor protein binds LDL and transports it into cells by endocytosis; 7 successive cysteine-rich repeats of about 40 amino acids are present in the N-terminal of this multidomain membrane protein; other homologous domains occur in related receptors, including the very low-density lipoprotein receptor and the LDL receptor-related protein/alpha 2-macroglobulin receptor, and in proteins which are functionally unrelated, such as the C9 component of complement; the binding of calcium is required for in vitro formation of the native disulfide isomer and is necessary in establishing and maintaining the modular structure
[3, 2, 5, 1, 4]

References

1.Three-dimensional structure of a cysteine-rich repeat from the low-density lipoprotein receptor. Daly NL, Scanlon MJ, Djordjevic JT, Kroon PA, Smith R. Proc. Natl. Acad. Sci. U.S.A. 92, 6334-8, (1995). View articlePMID: 7603991

2.Molecular basis of familial hypercholesterolaemia from structure of LDL receptor module. Fass D, Blacklow S, Kim PS, Berger JM. Nature 388, 691-3, (1997). View articlePMID: 9262405

3.LDLR Database (second edition): new additions to the database and the software, and results of the first molecular analysis. Varret M, Rabes JP, Thiart R, Kotze MJ, Baron H, Cenarro A, Descamps O, Ebhardt M, Hondelijn JC, Kostner GM, Miyake Y, Pocovi M, Schmidt H, Schuster H, Stuhrmann M, Yamamura T, Junien C, Beroud C, Boileau C. Nucleic Acids Res 26, 248-52, (1998). PMID: 9399845

4.The human LDL receptor: a cysteine-rich protein with multiple Alu sequences in its mRNA. Yamamoto T, Davis CG, Brown MS, Schneider WJ, Casey ML, Goldstein JL, Russell DW. Cell 39, 27-38, (1984). View articlePMID: 6091915

5.Protein folding and calcium binding defects arising from familial hypercholesterolemia mutations of the LDL receptor. Blacklow SC, Kim PS. Nat Struct Biol 3, 758-62, (1996). PMID: 8784348

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