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1                                              LAL can be specifically inhibited by a variety of 3,4-di
2                                              LAL defects cause Wolman disease (WD) and CE storage dis
3                                              LAL deficiency causes expansion of CD11b(+)Gr-1(+) immat
4                                              LAL deficiency impaired T cell development in the thymus
5                                              LAL is encoded by LIPA (10q23.31) and the most common mu
6                         A mouse model with a LAL null mutation was produced by targeting disruption o
7     Humans and mice with defective or absent LAL activity accumulate large amounts of cholesteryl est
8                                           An LAL null (lal-/-) mouse model closely mimics human WD/CE
9 , and 30 minutes using limulus lysate assay (LAL) and EndoCAb Ig assays.
10 cid (EDTA); limulus amoebocyte lysate assay (LAL); pertussis toxin (PTX); forward scatter (FSC); Inte
11 ed with the initial refractive target before LAL implantation.
12 stiffness in control strips to levels beyond LAL specimens.
13                     To test how myeloid cell LAL controls myelopoiesis and lymphopoiesis, a myeloid-s
14                                 Myeloid cell LAL expression improved the proliferation and function o
15 n the thymus, reconstitution of myeloid cell LAL restored development of thymocytes at the double-neg
16  using a commercially available colorimetric LAL assay.
17                          Patients completing LAL-CL01 were eligible to enroll in the extension study
18 o disease formation in various organs during LAL deficiency.
19                                    Following LAL, myocardial stresses at given strains and circumfere
20        These studies provide feasibility for LAL enzyme therapy in human WD and CESD.
21                   We present a mechanism for LAL inhibition by these compounds whereby LAL transientl
22 cal trials of enzyme replacement therapy for LAL deficiency are currently being developed.
23                                     ECs from LAL-deficient (lal(-/-)) mice possess enhanced prolifera
24  that metabolic reprogramming resulting from LAL deficiency enhances the ability of ECs to stimulate
25                                 Furthermore, LAL-deficient mice challenged with RE gavage exhibited l
26                                To assess how LAL in lung epithelial cells plays a role in this inflam
27               Comparisons of mouse and human LAL genes organization revealed identical intron/exon bo
28 nsgenic system was generated to induce human LAL (hLAL) expression in the lal-/- genetic background u
29         Myeloid-specific expression of human LAL (hLAL) in lal(-/-) mice rescues these malignant phen
30 yte, hepatocyte-specific expression of human LAL (hLAL) in lal(-/-) mice was established by cross-bre
31 epithelial cell-specific expression of human LAL (hLAL) in Lipa(-/-) mice was established by crossbre
32  effects and safety of the recombinant human LAL, sebelipase alfa, nine patients received four once-w
33 py was tested using mannose terminated human LAL expressed in Pichia pastoris (phLAL), purified, and
34 oligosaccharide chains was tested with human LAL expressed in Pichia pastoris (phLAL) and CHO cells (
35 rinsic expression of the lysosomal hydrolase LAL (lysosomal acid lipase) to mobilize FA for FAO and m
36                                           In LAL gene-knockout (lal(-/-)) mice, blockage of cholester
37 ekly infusions (0.35, 1, or 3 mg.kg(-1) ) in LAL-CL01, which is the first human study of this investi
38 e hypothesized that ECs are dysfunctional in LAL-deficient (lal(-/-)) mice.
39  MDSCs or mTOR to rejuvenate EC functions in LAL deficiency-related diseases.
40                   One major manifestation in LAL-deficient (Lipa(-/-)) mice is an increase of tumor g
41 ent, LV stresses and stiffness normalized in LAL specimens and microtubule density following colchici
42       Transaminases decreased in patients in LAL-CL01 and increased between studies.
43  density following colchicine was similar in LAL to control.
44 ceiving ongoing sebelipase alfa treatment in LAL-CL04, the mean +/- standard deviation (SD) decreases
45 t week 12 compared to the baseline values in LAL-CL01 were 46 +/- 21 U/L (-52%) and 21 +/- 14 U/L (-3
46                               Interestingly, LAL-deficient mice exhibited increased RE content in the
47 lysosomal acid lipase (hLAL) expression into LAL gene knockout (lal(-/-)) mice.
48 ly used for endotoxin level determination is LAL (Limulus Amebocyte Lysate) assay.
49 igh Risk Adult Acute Lymphoblastic Leukemia [LAL-AR/2003]) assigned adolescent and adult patients (ag
50 ton stage 27 following left atrial ligation (LAL) at stage 21 to reduce LV volume load and create lef
51 ding, CTB) or reduced (left atrial ligation, LAL) hemodynamic loading of the embryonic heart.
52                        Our observations link LAL to metabolic reprogramming in lymphocytes and show t
53                       Lysosomal acid lipase (LAL) cleaves cholesteryl esters and triglycerides to gen
54                       Lysosomal acid lipase (LAL) cleaves cholesteryl esters and triglycerides to gen
55 lying mechanisms that lysosomal acid lipase (LAL) deficiency causes infiltration of myeloid-derived s
56                       Lysosomal acid lipase (LAL) deficiency causes systemic expansion and infiltrati
57              Of note, lysosomal acid lipase (LAL) deficiency facilitates melanoma growth and metastas
58                       Lysosomal acid lipase (LAL) has been recently identified as a potential therape
59                       Lysosomal acid lipase (LAL) hydrolyzes cholesteryl esters and triglycerides to
60                       Lysosomal acid lipase (LAL) is a key enzyme that cleaves cholesteryl esters and
61                       Lysosomal acid lipase (LAL) is essential for the clearance of endocytosed chole
62                       Lysosomal acid lipase (LAL) is essential for the hydrolysis of cholesteryl este
63                       Lysosomal acid lipase (LAL) is essential for the hydrolysis of the triglyceride
64                       Lysosomal acid lipase (LAL) is required for the hydrolysis of intracellular cho
65                       Lysosomal acid lipase (LAL) is the critical enzyme for the hydrolysis of the tr
66                       Lysosomal acid lipase (LAL) is the critical enzyme for the hydrolysis of trigly
67 bsequent lipolysis by lysosomal acid lipase (LAL) was important for the engagement of elevated oxidat
68                       Lysosomal acid lipase (LAL), a key enzyme in the metabolic pathway of neutral l
69 herited deficiency of lysosomal acid lipase (LAL), is an underappreciated cause of progressive liver
70 deficient activity of lysosomal acid lipase (LAL).
71  lalistat, a specific lysosomal acid lipase (LAL/Lipa) inhibitor on LD degradation in HSCs during act
72         Deficiency of lysosomal acid lipase (LAL; official name Lipa, encoded by Lipa) in mice (lal(-
73 s we examined neonatal levator auris longus (LAL) and 4th deep lumbrical (4DL) muscles, as well as ad
74 ersus abdominis (TVA), levator auris longus (LAL) and lumbrical muscles were disrupted in both mouse
75                                 In the lung, LAL is highly expressed in alveolar type II epithelial c
76  as assayed in the Limulus amebocyte lysate (LAL) test.
77 ility to coagulate Limulus amebocyte lysate (LAL).
78 dotoxin using the limulus amebocyte lystate (LAL) gel clot method.
79          In this study, we found that murine LAL exhibits RE hydrolase activity.
80 zygote knockout mice (lal -/lal-) produce no LAL mRNA, protein or enzyme activity.
81 +/lal- mice have approximately 50% of normal LAL activity and do not show lipid accumulation.
82                           Through week 12 of LAL-CL04, these seven patients also showed mean decrease
83    The unparalleled recognition abilities of LAL biosensors perched with remarkable sensitivity, high
84 acological inhibition or genetic ablation of LAL in murine liver largely reduced in vitro acid RE hyd
85 urring in the tested sample upon addition of LAL.
86                     Western blot analysis of LAL embryos showed an increase in both total and polymer
87                           The coagulation of LAL is commonly used to signal the presence of endotoxin
88         To understand that the expression of LAL mRNA and protein is tissue and cell specifically reg
89                Pharmacological inhibition of LAL in the human hepatocyte cell line HepG2, incubated w
90 mides, esters, and ketones for inhibition of LAL.
91 azole carbamates are effective inhibitors of LAL.
92               To test the functional role of LAL in hepatocyte, hepatocyte-specific expression of hum
93 ice provide a model to determine the role of LAL in lipid metabolism and the pathogenesis of its defi
94 also contain retinyl esters (REs), a role of LAL in the clearance of endocytosed REs has not been rep
95     These results indicate a crucial role of LAL-regulated mTOR signaling in the production and funct
96 only approximately 3%-5% of normally spliced LAL.
97 e eligible to enroll in the extension study (LAL-CL04) in which they again received four once-weekly
98  with a history of laser refractive surgery, LAL implantation and postimplantation adjustment provide
99         These results support a concept that LAL in hepatocytes is a critical metabolic enzyme in con
100         These results support a concept that LAL is a critical metabolic enzyme in lung epithelial ce
101               These studies demonstrate that LAL in myeloid cells plays a critical role in maintainin
102          These results provide evidence that LAL is an important regulator of myelopoiesis during hem
103                    Our results indicate that LAL has a critical role in regulating MDSCs' ability to
104        In summary, our results indicate that LAL is the major acid RE hydrolase and required for func
105                    Our results indicate that LAL regulates EC functions through interaction with MDSC
106 dial circulating RE content, indicating that LAL is required for efficient nutritional vitamin A avai
107                        Our data suggest that LAL/Lipa is involved in the degradation of a specific pr
108                                          The LAL endotoxin detection limit for samples dispersed in C
109  liposomes, but did not appear to affect the LAL assay sensitivity once the liposomes were completely
110 t not DSPG, at 10 mol% further decreased the LAL endotoxin detection limit.
111               Mice double homozygous for the LAL and MMR deficiences (lal-/-;MMR-/-) showed phLAL upt
112                                       In the LAL-deficient (lal(-/-)) mouse model, melanoma metastasi
113 L to consume unpolymerized macromeres in the LAL.
114  ultraviolet light to alter the shape of the LAL and hence its refractive power.
115 solubilization and/or the sensitivity of the LAL assay.
116                 A targeted disruption of the LAL locus produced a null (lal( -/-)) mouse model that m
117 tor neurons in the caudal muscle band of the LAL.
118 ents, we applied 2 lock-in treatments to the LAL to consume unpolymerized macromeres in the LAL.
119 ation in HSCs during activation in vitro The LAL inhibitor increased the levels of TAG, cholesteryl e
120 l refractive surgery were implanted with the LAL during cataract surgery performed at a single clinic
121                                   Therefore, LAL, its downstream genes, and lipid mediators all play
122 ral TVA muscle compared with the fast-twitch LAL and lumbrical muscles.
123 re HPS function relative to controls whereas LAL was associated with delayed conversion to apical ini
124 or LAL inhibition by these compounds whereby LAL transiently carbamoylates the enzyme similarly to pr
125 aching significance (P=0.061) were seen with LAL only.

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