-
1
-
-
0000600880
-
Familial hypercholesterolemia
-
C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, NY
-
Goldstein, J. L., H. H. Hobbs, and M. S. Brown. 2001. Familial hypercholesterolemia. In The Metabolic and Molecular Basis of Inherited Disease. C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle, editors. McGraw-Hill, NY. 2863-2914.
-
(2001)
The Metabolic and Molecular Basis of Inherited Disease
, pp. 2863-2914
-
-
Goldstein, J.L.1
Hobbs, H.H.2
Brown, M.S.3
-
2
-
-
70449368195
-
The unique role of proprotein convertase subtilisin/kexin 9 in cholesterol homeostasis
-
Mousavi, S. A., K. E. Berge, and T. P. Leren. 2009. The unique role of proprotein convertase subtilisin/kexin 9 in cholesterol homeostasis. J. Intern. Med. 266:507-519.
-
(2009)
J. Intern. Med.
, vol.266
, pp. 507-519
-
-
Mousavi, S.A.1
Berge, K.E.2
Leren, T.P.3
-
3
-
-
0037417807
-
The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): Liver regeneration and neuronal differentiation
-
DOI 10.1073/pnas.0335507100
-
Seidah, N. G., S. Benjannet, L. Wickham, J. Marcinkiewicz, S. B. Jasmin, S. Stifani, A. Basak, A. Prat, and M. Chrétien. 2003. The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): liver regeneration and neuronal differentiation. Proc. Natl. Acad. Sci. USA. 100:928-933. (Pubitemid 36183932)
-
(2003)
Proceedings of the National Academy of Sciences of the United States of America
, vol.100
, Issue.3
, pp. 928-933
-
-
Seidah, N.G.1
Benjannet, S.2
Wickham, L.3
Marcinkiewicz, J.4
Belanger, J.S.5
Stifani, S.6
Basak, A.7
Prat, A.8
Chretien, M.9
-
4
-
-
34547108600
-
Binding of proprotein convertase subtilisin/kexin type 9 to epidermal growth factor-like repeat A of low density lipoprotein receptor decreases receptor recycling and increases degradation
-
DOI 10.1074/jbc.M702027200
-
Zhang, D. W., T. A. Lagace, R. Garuti, Z. Zhao, M. McDonald, J. D. Horton, J. C. Cohen, and H. H. Hobbs. 2007. Binding of proprotein convertase subtilisin/kexin type 9 to epidermal growth factor-like repeat A of low density lipoprotein receptor decreases receptor recycling and increases degradation. J. Biol. Chem. 282:18602-18612. (Pubitemid 47100234)
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.25
, pp. 18602-18612
-
-
Zhang, D.-W.1
Lagace, T.A.2
Garuti, R.3
Zhao, Z.4
McDonald, M.5
Horton, J.D.6
Cohen, J.C.7
Hobbs, H.H.8
-
5
-
-
34247605063
-
Structural and biophysical studies of PCSK9 and its mutants linked to familial hypercholesterolemia
-
DOI 10.1038/nsmb1235, PII NSMB1235
-
Cunningham, D., D. E. Danley, K. F. Geoghegan, M. C. Griffor, J. L. Hawkins, T. A. Subashi, A. H. Varghese, M. J. Ammirati, J. S. Culp, L. R. Hoth, et al. 2007. Structural and biophysical studies of PCSK9 and its mutants linked to familial hypercholesterolemia. Nat. Struct. Mol. Biol. 14:413-419. (Pubitemid 46685885)
-
(2007)
Nature Structural and Molecular Biology
, vol.14
, Issue.5
, pp. 413-419
-
-
Cunningham, D.1
Danley, D.E.2
Geoghegan, K.F.3
Griffor, M.C.4
Hawkins, J.L.5
Subashi, T.A.6
Varghese, A.H.7
Ammirati, M.J.8
Culp, J.S.9
Hoth, L.R.10
Mansour, M.N.11
McGrath, K.M.12
Seddon, A.P.13
Shenolikar, S.14
Stutzman-Engwall, K.J.15
Warren, L.C.16
Xia, D.17
Qiu, X.18
-
6
-
-
34247892364
-
The Crystal Structure of PCSK9: A Regulator of Plasma LDL-Cholesterol
-
DOI 10.1016/j.str.2007.04.004, PII S0969212607001463
-
Piper, D. E., S. Jackson, Q. Liu, W. G. Romanow, S. Shetterly, S. T. Thibault, B. Shan, and N. P. C. Walker. 2007. The crystal structure of PCSK9: a regulator of plasma LDL-cholesterol. Structure. 15:545-552. (Pubitemid 46702689)
-
(2007)
Structure
, vol.15
, Issue.5
, pp. 545-552
-
-
Piper, D.E.1
Jackson, S.2
Liu, Q.3
Romanow, W.G.4
Shetterly, S.5
Thibault, S.T.6
Shan, B.7
Walker, N.P.C.8
-
7
-
-
41149133845
-
Molecular basis for LDL receptor recognition by PCSK9
-
DOI 10.1073/pnas.0712064105
-
Kwon, H. J., T. A. Lagace, M. C. McNutt, J. D. Horton, and J. Deisenhofer. 2008. Molecular basis for LDL receptor recognition by PCSK9. Proc. Natl. Acad. Sci. USA. 105:1820-1825. (Pubitemid 351439423)
-
(2008)
Proceedings of the National Academy of Sciences of the United States of America
, vol.105
, Issue.6
, pp. 1820-1825
-
-
Hyock, J.K.1
Lagace, T.A.2
McNutt, M.C.3
Horton, J.D.4
Deisenhofer, J.5
-
8
-
-
48249115641
-
Investigations on the evolutionary conservation of PCSK9 reveal a functionally important protrusion
-
Cameron, J., Ø. L. Holla, K. E. Berge, M. A. Kulseth, T. Ranheim, T. P. Leren, and J. K. Laerdahl. 2008. Investigations on the evolutionary conservation of PCSK9 reveal a functionally important protrusion. FEBS J. 275:4121-4133.
-
(2008)
FEBS J.
, vol.275
, pp. 4121-4133
-
-
Cameron, J.1
Holla, Ø.L.2
Berge, K.E.3
Kulseth, M.A.4
Ranheim, T.5
Leren, T.P.6
Laerdahl, J.K.7
-
9
-
-
51349161358
-
Structural requirements for PCSK9-mediated degradation of the low-density lipoprotein receptor
-
Zhang, D. W., R. Garuti, W. J. Tang, J. C. Cohen, and H. H. Hobbs. 2008. Structural requirements for PCSK9-mediated degradation of the low-density lipoprotein receptor. Proc. Natl. Acad. Sci. USA. 105:13045-13050.
-
(2008)
Proc. Natl. Acad. Sci. USA.
, vol.105
, pp. 13045-13050
-
-
Zhang, D.W.1
Garuti, R.2
Tang, W.J.3
Cohen, J.C.4
Hobbs, H.H.5
-
10
-
-
78650904300
-
A PCSK9-binding antibody that structurally mimics the EGF(A) domain of LDL-receptor reduces LDL cholesterol in vivo
-
Ni, Y. G., S. Di Marco, J. H. Condra, L. B. Peterson, W. Wang, F. Wang, S. Pandit, H. A. Hammond, R. Rosa, R. T. Cummings, et al. 2011. A PCSK9-binding antibody that structurally mimics the EGF(A) domain of LDL-receptor reduces LDL cholesterol in vivo. J. Lipid Res. 52:78-86.
-
(2011)
J. Lipid Res.
, vol.52
, pp. 78-86
-
-
Ni, Y.G.1
Di Marco, S.2
Condra, J.H.3
Peterson, L.B.4
Wang, W.5
Wang, F.6
Pandit, S.7
Hammond, H.A.8
Rosa, R.9
Cummings, R.T.10
-
11
-
-
79953136043
-
A two-step binding model of PCSK9 interaction with the low density lipoprotein receptor
-
Yamamoto, T., C. Lu, and R. O. Ryan. 2011. A two-step binding model of PCSK9 interaction with the low density lipoprotein receptor. J. Biol. Chem. 286:5464-5470.
-
(2011)
J. Biol. Chem.
, vol.286
, pp. 5464-5470
-
-
Yamamoto, T.1
Lu, C.2
Ryan, R.O.3
-
12
-
-
79952454325
-
Removal of acidic residues of the prodomain increases its activity towards the LDL receptor
-
Holla, Ø. L., J. K. Laerdahl, T. B. Strøm, K. Tveten, J. Cameron, K. E. Berge, and T. P. Leren. 2011. Removal of acidic residues of the prodomain increases its activity towards the LDL receptor. Biochem. Biophys. Res. Commun. 406:234-238.
-
(2011)
Biochem. Biophys. Res. Commun.
, vol.406
, pp. 234-238
-
-
Holla, Ø.L.1
Laerdahl, J.K.2
Strøm, T.B.3
Tveten, K.4
Cameron, J.5
Berge, K.E.6
Leren, T.P.7
-
13
-
-
33947134366
-
Degradation of the LDL receptors by PCSK9 is not mediated by a secreted protein acted upon by PCSK9 extracellularly
-
Holla, Ø. L., J. Cameron, K. E. Berge, T. Ranheim, and T. P. Leren. 2007. Degradation of the LDL receptors by PCSK9 is not mediated by a secreted protein acted upon by PCSK9 extracellularly. BMC Cell Biol. 8:9.
-
(2007)
BMC Cell Biol.
, vol.8
, pp. 9
-
-
Holla, Ø.L.1
Cameron, J.2
Berge, K.E.3
Ranheim, T.4
Leren, T.P.5
-
14
-
-
33644863652
-
Retention of mutant low density lipoprotein receptor in endoplasmic reticulum (ER) leads to ER stress
-
DOI 10.1074/jbc.M507071200
-
Sørensen, S., T. Ranheim, K. S. Bakken, T. P. Leren, and M. A. Kulseth. 2006. Retention of mutant low density lipoprotein receptor in endoplasmic reticulum (ER) leads to ER stress. J. Biol. Chem. 281:468-476. (Pubitemid 43671209)
-
(2006)
Journal of Biological Chemistry
, vol.281
, Issue.1
, pp. 468-476
-
-
Sorensen, S.1
Ranheim, T.2
Bakken, K.S.3
Leren, T.P.4
Kulseth, M.A.5
-
15
-
-
33646199829
-
Effect of mutations in the PCSK9 gene on the cell surface LDL receptors
-
Cameron, J., Ø. L. Holla, T. Ranheim, M. A. Kulseth, K. E. Berge, and T. P. Leren. 2006. Effect of mutations in the PCSK9 gene on the cell surface LDL receptors. Hum. Mol. Genet. 15:1551-1558.
-
(2006)
Hum. Mol. Genet.
, vol.15
, pp. 1551-1558
-
-
Cameron, J.1
Holla, Ø.L.2
Ranheim, T.3
Kulseth, M.A.4
Berge, K.E.5
Leren, T.P.6
-
16
-
-
73749086245
-
A chimeric LDL receptor containing the cytoplasmic domain of the transferrin receptor is degraded by PCSK9
-
Holla, Ø. L., T. B. Strøm, J. Cameron, K. E. Berge, and T. P. Leren. 2010. A chimeric LDL receptor containing the cytoplasmic domain of the transferrin receptor is degraded by PCSK9. Mol. Genet. Metab. 99:149-156.
-
(2010)
Mol. Genet. Metab.
, vol.99
, pp. 149-156
-
-
Holla, Ø.L.1
Strøm, T.B.2
Cameron, J.3
Berge, K.E.4
Leren, T.P.5
-
17
-
-
57349090665
-
a values for protein-ligand complexes
-
DOI 10.1002/prot.22102
-
Bas, D. C., D. M. Rogers, and J. H. Jensen. 2008. Very fast prediction and rationalization of pKa values for protein-ligand complexes. Proteins. 73:765-783. (Pubitemid 352788657)
-
(2008)
Proteins: Structure, Function and Genetics
, vol.73
, Issue.3
, pp. 765-783
-
-
Bas, D.C.1
Rogers, D.M.2
Jensen, J.H.3
-
19
-
-
78650361274
-
Effects of the prosegment and pH on the activity of PCSK9: Evidence for additional processing events
-
Benjannet, S., Y. G. Saavedra, J. Hamelin, M. C. Asselin, R. Essalmani, A. Pasquato, P. Lemaire, G. Duke, B. Miao, F. Duclos, et al. 2010. Effects of the prosegment and pH on the activity of PCSK9: evidence for additional processing events. J. Biol. Chem. 285:40965-40978.
-
(2010)
J. Biol. Chem.
, vol.285
, pp. 40965-40978
-
-
Benjannet, S.1
Saavedra, Y.G.2
Hamelin, J.3
Asselin, M.C.4
Essalmani, R.5
Pasquato, A.6
Lemaire, P.7
Duke, G.8
Miao, B.9
Duclos, F.10
-
20
-
-
0037147281
-
Structure of the LDL receptor extracellular domain at endosomal pH
-
DOI 10.1126/science.1078124
-
Rudenko, G., L. Henry, K. Henderson, K. Ichtchenko, M. S. Brown, J. L. Goldstein, and J. Deisenhofer. 2002. Structure of the LDL receptor extracellular domain at endosomal pH. Science. 298:2353-2358. (Pubitemid 36014201)
-
(2002)
Science
, vol.298
, Issue.5602
, pp. 2353-2358
-
-
Rudenko, G.1
Henry, L.2
Henderson, K.3
Ichtchenko, K.4
Brown, M.S.5
Goldstein, J.L.6
Deisenhofer, J.7
-
21
-
-
40349113279
-
Characterization of novel mutations in the catalytic domain of the PCSK9 gene
-
DOI 10.1111/j.1365-2796.2007.01915.x
-
Cameron, J., Ø. L. Holla, J. K. Laerdahl, M. A. Kulseth, T. Ranheim, T. Rognes, K. E. Berge, and T. P. Leren. 2008. Characterization of novel mutations in the catalytic domain of the PCSK9 gene. J. Intern. Med. 263:420-431. (Pubitemid 351342682)
-
(2008)
Journal of Internal Medicine
, vol.263
, Issue.4
, pp. 420-431
-
-
Cameron, J.1
Holla, O.L.2
Laerdahl, J.K.3
Kulseth, M.A.4
Ranheim, T.5
Rognes, T.6
Berge, K.E.7
Leren, T.P.8
-
23
-
-
0026056610
-
Anomalous behaviour of a protein during SDS/PAGE corrected by chemical modification of carboxylic groups
-
Matagne, A., B. Joris, and J. M. Frère. 1991. Anomalous behaviour of a protein during SDS/PAGE corrected by chemical modification of carboxylic groups. Biochem. J. 280:553-556.
-
(1991)
Biochem. J.
, vol.280
, pp. 553-556
-
-
Matagne, A.1
Joris, B.2
Frère, J.M.3
|