Expression of LDLRs (Low-Density Lipoprotein Receptors), Dyslipidemia Severity, and Response to PCSK9 (Proprotein Convertase Subtilisin Kexin Type 9) Inhibition in Homozygous Familial Hypercholesterolemia

Expression of LDLRs (Low-Density Lipoprotein Receptors), Dyslipidemia Severity, and Response to... Editorial Expression of LDLRs (Low-Density Lipoprotein Receptors), Dyslipidemia Severity, and Response to PCSK9 (Proprotein Convertase Subtilisin Kexin Type 9) Inhibition in Homozygous Familial Hypercholesterolemia Connecting the Dots Raul D. Santos omozygous familial hypercholesterolemia (HoFH) is LDL-C in HoFH, it is uncertain whether this is affected by 4–7 Hcharacterized by extremely elevated low-density lipo- LDLR expression. In theory, LDLR expression and func- protein cholesterol (LDL-C) levels (usually 4–5-fold normal) tion should not influence LDL-C lowering by mipomersen and appearance of xanthomas, aggressive atherosclerotic car- (an apoB synthesis inhibitor) or lomitapide (a microsomal diovascular as well as aortic and supra-aortic valve diseases, triglyceride transfer protein inhibitor) that reduce production before the age of 20 years. The severity of the HoFH phe- of LDL precursors (Figure), and this seems to be the case from notype and its ominous consequences correlate with LDL-C small numbers of patients in clinical trials treated with these levels. The latter are influenced primarily by the type of famil- newer agents. ial hypercholesterolemia (FH)-causing molecular defect. The Monoclonal antibodies against PCSK9, like alirocumab gravest phenotypes result from homozygous or compound and evolocumab, reduce PCSK9-mediated LDLR degrada- heterozygous mutations in the LDLR gene (low-density lipo- tion and consequently lower http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Arteriosclerosis, Thrombosis, and Vascular Biology Wolters Kluwer Health

Expression of LDLRs (Low-Density Lipoprotein Receptors), Dyslipidemia Severity, and Response to PCSK9 (Proprotein Convertase Subtilisin Kexin Type 9) Inhibition in Homozygous Familial Hypercholesterolemia

Loading next page...
 
/lp/wolters_kluwer/expression-of-ldlrs-low-density-lipoprotein-receptors-dyslipidemia-s8Ufv1c6Y0
Publisher
Wolters Kluwer
Copyright
© 2018 American Heart Association, Inc.
ISSN
1079-5642
eISSN
1524-4636
D.O.I.
10.1161/ATVBAHA.117.310675
Publisher site
See Article on Publisher Site

Abstract

Editorial Expression of LDLRs (Low-Density Lipoprotein Receptors), Dyslipidemia Severity, and Response to PCSK9 (Proprotein Convertase Subtilisin Kexin Type 9) Inhibition in Homozygous Familial Hypercholesterolemia Connecting the Dots Raul D. Santos omozygous familial hypercholesterolemia (HoFH) is LDL-C in HoFH, it is uncertain whether this is affected by 4–7 Hcharacterized by extremely elevated low-density lipo- LDLR expression. In theory, LDLR expression and func- protein cholesterol (LDL-C) levels (usually 4–5-fold normal) tion should not influence LDL-C lowering by mipomersen and appearance of xanthomas, aggressive atherosclerotic car- (an apoB synthesis inhibitor) or lomitapide (a microsomal diovascular as well as aortic and supra-aortic valve diseases, triglyceride transfer protein inhibitor) that reduce production before the age of 20 years. The severity of the HoFH phe- of LDL precursors (Figure), and this seems to be the case from notype and its ominous consequences correlate with LDL-C small numbers of patients in clinical trials treated with these levels. The latter are influenced primarily by the type of famil- newer agents. ial hypercholesterolemia (FH)-causing molecular defect. The Monoclonal antibodies against PCSK9, like alirocumab gravest phenotypes result from homozygous or compound and evolocumab, reduce PCSK9-mediated LDLR degrada- heterozygous mutations in the LDLR gene (low-density lipo- tion and consequently lower

Journal

Arteriosclerosis, Thrombosis, and Vascular BiologyWolters Kluwer Health

Published: Mar 1, 2018

There are no references for this article.

You’re reading a free preview. Subscribe to read the entire article.


DeepDyve is your
personal research library

It’s your single place to instantly
discover and read the research
that matters to you.

Enjoy affordable access to
over 18 million articles from more than
15,000 peer-reviewed journals.

All for just $49/month

Explore the DeepDyve Library

Search

Query the DeepDyve database, plus search all of PubMed and Google Scholar seamlessly

Organize

Save any article or search result from DeepDyve, PubMed, and Google Scholar... all in one place.

Access

Get unlimited, online access to over 18 million full-text articles from more than 15,000 scientific journals.

Your journals are on DeepDyve

Read from thousands of the leading scholarly journals from SpringerNature, Elsevier, Wiley-Blackwell, Oxford University Press and more.

All the latest content is available, no embargo periods.

See the journals in your area

DeepDyve

Freelancer

DeepDyve

Pro

Price

FREE

$49/month
$360/year

Save searches from
Google Scholar,
PubMed

Create lists to
organize your research

Export lists, citations

Read DeepDyve articles

Abstract access only

Unlimited access to over
18 million full-text articles

Print

20 pages / month

PDF Discount

20% off