Adam M Lopez
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Explore the profile of Adam M Lopez including associated specialties, affiliations and a list of published articles.
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20
Citations
143
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Recent Articles
1.
Aqul A, Ramirez C, Lopez A, Burns D, Repa J, Turley S
Lipids
. 2021 Oct;
57(1):3-16.
PMID: 34618372
Lysosomal acid lipase (LAL), encoded by the gene LIPA, facilitates the intracellular processing of lipids by hydrolyzing cholesteryl esters and triacylglycerols present in newly internalized lipoproteins. Loss-of-function mutations in LIPA...
2.
Ramirez C, Taylor A, Lopez A, Repa J, Turley S
Steroids
. 2020 Sep;
164:108725.
PMID: 32890578
Lipids present in lipoproteins cleared from the circulation are processed sequentially by three major proteins within the late endosomal/lysosomal (E/L) compartment of all cells: lysosomal acid lipase (LAL), Niemann-Pick (NPC)...
3.
Lopez A, Ramirez C, Taylor A, Jones R, Repa J, Turley S
Dig Dis Sci
. 2019 Jul;
65(1):158-167.
PMID: 31312996
Background: Mutations in the NPC1 gene result in sequestration of unesterified cholesterol (UC) and glycosphingolipids in most tissues leading to multi-organ disease, especially in the brain, liver, lungs, and spleen....
4.
Lopez A, Jones R, Repa J, Turley S
Am J Physiol Gastrointest Liver Physiol
. 2018 Jun;
315(4):G454-G463.
PMID: 29878847
Cholesteryl esters are generated at multiple sites in the body by sterol O-acyltransferase (SOAT) 1 or SOAT2 in various cell types and lecithin cholesterol acyltransferase in plasma. Esterified cholesterol and...
5.
Lutjohann D, Lopez A, Chuang J, Kerksiek A, Turley S
Lipids
. 2018 May;
53(4):363-373.
PMID: 29770459
Rett syndrome (RS) is a pervasive neurodevelopmental disorder resulting from loss-of-function mutations in the X-linked gene methyl-Cpg-binding protein 2 (MECP2). Using a well-defined model for RS, the C57BL6/Mecp2 mouse, we...
6.
Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse
Lopez A, Chuang J, Turley S
Steroids
. 2017 Dec;
130:7-14.
PMID: 29246491
Although only a small proportion of cholesterol in the body is esterified, in several diseases marked expansion of the esterified cholesterol (EC) pool occurs. These include Wolman disease (WD) and...
7.
Chuang J, Lopez A, Turley S
Biochem Pharmacol
. 2017 Mar;
135:116-125.
PMID: 28322747
Esterified cholesterol (EC) and triglycerides, contained within lipoproteins taken up by cells, are hydrolysed by lysosomal acid lipase (LAL) in the late endosomal/lysosomal (E/L) compartment. The resulting unesterified cholesterol (UC)...
8.
Lopez A, Chuang J, Turley S
Methods Mol Biol
. 2017 Feb;
1583:241-256.
PMID: 28205179
Every organ in the body is capable of synthesizing cholesterol de novo but at rates that vary with a constellation of factors. A significant proportion of the hydrogen atoms present...
9.
Lopez A, Chuang J, Posey K, Turley S
Brain Res
. 2016 Dec;
1657:383.
PMID: 28017700
No abstract available.
10.
Lopez A, Chuang J, Posey K, Turley S
Brain Res
. 2016 Oct;
1654(Pt A):77-84.
PMID: 27789278
Mutations in the X-linked gene methyl-CpG-binding protein 2 (MECP2) are the principal cause of Rett syndrome, a progressive neurodevelopmental disorder afflicting 1 in 10,000 to 15,000 females. Studies using hemizygous...