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 LAL is the standard reagent to test for endotoxin contam
7 LAL overexpression increased levels of BAT free choleste
8 A mouse model with
a LAL null mutation was produced by targeting disruption o
9 Humans and mice with defective or
absent LAL activity accumulate large amounts of cholesteryl est
10 clusion, our study demonstrates that
adipose LAL drives tissue-cholesterol homeostasis and affects BA
11 An LAL null (lal-/-) mouse model closely mimics human WD/CE
12 a result, we identified SAOUHSC_02373 as
an LAL with high selectivity for L-aspartate and L-methioni
13 me proliferator-activated receptor gamma,
an LAL downstream effector, reduces lal(-/-) Treg and Breg
14 xisome proliferator-activated receptor y,
an LAL downstream effector, reduces lal(-/-) Treg and Breg
15 Combined inhibition of ATGL
and LAL resulted in large LDs, suggesting that lipolysis tar
16 A and LPS were quantified with LTA-ELISA
and LAL assay, respectively.
17 nverge on the same genetic subset of FSB
and LAL neurons.
18 , and 30 minutes using limulus lysate
assay (
LAL) and EndoCAb Ig assays.
19 cid (EDTA); limulus amoebocyte lysate
assay (
LAL); pertussis toxin (PTX); forward scatter (FSC); Inte
20 ed with the initial refractive target
before LAL implantation.
21 ffects BAT metabolism, suggesting
beneficial LAL activation in anti-obesity approaches aimed at react
22 stiffness in control strips to levels
beyond LAL specimens.
23 emical pathways of nanocrystals generated
by LAL, providing a rational guideline for the conscious pr
24 To test how myeloid
cell LAL controls myelopoiesis and lymphopoiesis, a myeloid-s
25 Myeloid
cell LAL expression improved the proliferation and function o
26 n the thymus, reconstitution of myeloid
cell LAL restored development of thymocytes at the double-neg
27 using a commercially available
colorimetric LAL assay.
28 Patients
completing LAL-CL01 were eligible to enroll in the extension study
29 sue (ATs), from obese patients has
decreased LAL expression compared with that from nonobese people.
30 Lysosomal acid lipase
deficiency (
LAL-D) is caused by mutations in the LIPA gene, which en
31 o disease formation in various organs
during LAL deficiency.
32 rscAAVrh74.LP1.LIPA treatment
elevated LAL enzyme activity above wild-type levels in all tissue
33 Following LAL, myocardial stresses at given strains and circumfere
34 were found in all ethnic groups, except
for LAL in the Kinh and Thai groups, and LP1L in the Tay gro
35 These studies provide feasibility
for LAL enzyme therapy in human WD and CESD.
36 We present a mechanism
for LAL inhibition by these compounds whereby LAL transientl
37 cal trials of enzyme replacement therapy
for LAL deficiency are currently being developed.
38 elop a curative single-treatment therapy
for LAL-D using adeno-associated virus (AAV).
39 tential to be a transformative treatment
for LAL-D.
40 ECs
from LAL-deficient (lal(-/-)) mice possess enhanced prolifera
41 that metabolic reprogramming resulting
from LAL deficiency enhances the ability of ECs to stimulate
42 Furthermore,
LAL-deficient mice challenged with RE gavage exhibited l
43 On a standard chow diet,
hep-
LAL-ko mice exhibited increased hepatic CE accumulation
44 ral lipid ester phenotype in livers from
hep-
LAL-ko mice indicates that LAL is limiting for CE turnov
45 specifically lacking LAL in hepatocytes (
hep-
LAL-ko).
46 sosome-enriched fractions from livers of
hep-
LAL-ko mice upon VitA/HFD feeding.
47 Feeding the
hep-
LAL-ko mice a vitamin A excess/high-fat diet (VitA/HFD)
48 To assess
how LAL in lung epithelial cells plays a role in this inflam
49 Comparisons of mouse and
human LAL genes organization revealed identical intron/exon bo
50 nsgenic system was generated to induce
human LAL (hLAL) expression in the lal-/- genetic background u
51 Myeloid-specific expression of
human LAL (hLAL) in lal(-/-) mice rescues these malignant phen
52 yte, hepatocyte-specific expression of
human LAL (hLAL) in lal(-/-) mice was established by cross-bre
53 epithelial cell-specific expression of
human LAL (hLAL) in Lipa(-/-) mice was established by crossbre
54 effects and safety of the recombinant
human LAL, sebelipase alfa, nine patients received four once-w
55 py was tested using mannose terminated
human LAL expressed in Pichia pastoris (phLAL), purified, and
56 oligosaccharide chains was tested with
human LAL expressed in Pichia pastoris (phLAL) and CHO cells (
57 rinsic expression of the lysosomal
hydrolase LAL (lysosomal acid lipase) to mobilize FA for FAO and m
58 In LAL gene-knockout (lal(-/-)) mice, blockage of cholester
59 ekly infusions (0.35, 1, or 3 mg.kg(-1) )
in LAL-CL01, which is the first human study of this investi
60 e hypothesized that ECs are dysfunctional
in LAL-deficient (lal(-/-)) mice.
61 MDSCs or mTOR to rejuvenate EC functions
in LAL deficiency-related diseases.
62 One major manifestation
in LAL-deficient (Lipa(-/-)) mice is an increase of tumor g
63 ent, LV stresses and stiffness normalized
in LAL specimens and microtubule density following colchici
64 Transaminases decreased in patients
in LAL-CL01 and increased between studies.
65 density following colchicine was similar
in LAL to control.
66 ceiving ongoing sebelipase alfa treatment
in LAL-CL04, the mean +/- standard deviation (SD) decreases
67 t week 12 compared to the baseline values
in LAL-CL01 were 46 +/- 21 U/L (-52%) and 21 +/- 14 U/L (-3
68 Interestingly,
LAL-deficient mice exhibited increased RE content in the
69 lysosomal acid lipase (hLAL) expression
into LAL gene knockout (lal(-/-)) mice.
70 ly used for endotoxin level determination
is LAL (Limulus Amebocyte Lysate) assay.
71 Mice globally
lacking LAL accumulate CE most prominently in the liver.
72 actions from livers of mice globally
lacking LAL, we detected residual acid hydrolytic activities aga
73 iver, we generated mice specifically
lacking LAL in hepatocytes (hep-LAL-ko).
74 h (LM2W); 7.06 mm for lower anterior
length (
LAL); 26.87 mm for lower posterior length 1 (LP1L); and
75 igh Risk Adult Acute Lymphoblastic
Leukemia [
LAL-AR/2003]) assigned adolescent and adult patients (ag
76 ton stage 27 following left atrial
ligation (
LAL) at stage 21 to reduce LV volume load and create lef
77 ding, CTB) or reduced (left atrial
ligation,
LAL) hemodynamic loading of the embryonic heart.
78 dulto (GIMEMA) Leucemia Acuta
Linfoblastica (
LAL) 2317 protocol investigated the frontline chemothera
79 Our observations
link LAL to metabolic reprogramming in lymphocytes and show t
80 ng dependency on lysosomal acid lipase (
LIPA/
LAL) and the microphthalmia/transcription factor E (MiT/
81 Lysosomal acid
lipase (
LAL) cleaves cholesteryl esters and triglycerides to gen
82 lying mechanisms that lysosomal acid
lipase (
LAL) deficiency causes infiltration of myeloid-derived s
83 Lysosomal acid
lipase (
LAL) deficiency causes systemic expansion and infiltrati
84 Of note, lysosomal acid
lipase (
LAL) deficiency facilitates melanoma growth and metastas
85 Lysosomal acid
lipase (
LAL) has been recently identified as a potential therape
86 Lysosomal acid
lipase (
LAL) hydrolyzes cholesteryl ester (CE) and retinyl ester
87 Lysosomal acid
lipase (
LAL) hydrolyzes cholesteryl esters and triglycerides to
88 Lysosomal acid
lipase (
LAL) is a critical lipid hydrolase that generates free f
89 Lysosomal acid
lipase (
LAL) is a key enzyme that cleaves cholesteryl esters and
90 Lysosomal acid
lipase (
LAL) is essential for the clearance of endocytosed chole
91 Lysosomal acid
lipase (
LAL) is essential for the hydrolysis of cholesteryl este
92 Lysosomal acid
lipase (
LAL) is essential for the hydrolysis of the triglyceride
93 Lysosomal acid
lipase (
LAL) is required for the hydrolysis of intracellular cho
94 Lysosomal acid
lipase (
LAL) is the critical enzyme for the hydrolysis of the tr
95 Lysosomal acid
lipase (
LAL) is the critical enzyme for the hydrolysis of trigly
96 bsequent lipolysis by lysosomal acid
lipase (
LAL) was important for the engagement of elevated oxidat
97 Lysosomal acid
lipase (
LAL), a key enzyme in the metabolic pathway of neutral l
98 the metabolic role of lysosomal acid
lipase (
LAL), highly expressed in adipocytes, is unclear.
99 herited deficiency of lysosomal acid
lipase (
LAL), is an underappreciated cause of progressive liver
100 anscriptional target, lysosomal acid
lipase (
LAL).
101 deficient activity of lysosomal acid
lipase (
LAL).
102 lalistat, a specific lysosomal acid
lipase (
LAL/Lipa) inhibitor on LD degradation in HSCs during act
103 Deficiency of lysosomal acid
lipase (
LAL; official name Lipa, encoded by Lipa) in mice (lal(-
104 technologies, with laser ablation in
liquid (
LAL) being a remarkable technique for their synthesis.
105 ovide input from the lateral accessory
lobe (
LAL) to the noduli (NO).
106 rons in the premotor lateral accessory
lobe (
LAL), we gained genetic access to a subset of these neur
107 body (FSB), and the lateral accessory
lobe (
LAL).
108 s we examined neonatal levator auris
longus (
LAL) and 4th deep lumbrical (4DL) muscles, as well as ad
109 ersus abdominis (TVA), levator auris
longus (
LAL) and lumbrical muscles were disrupted in both mouse
110 In the
lung,
LAL is highly expressed in alveolar type II epithelial c
111 as assayed in the Limulus amebocyte
lysate (
LAL) test.
112 parallelization of Limulus amebocyte
lysate (
LAL)-based bacterial endotoxin testing using centrifugal
113 ility to coagulate Limulus amebocyte
lysate (
LAL).
114 netic chromogenic limulus amoebocyte
lysate (
LAL) assay.
115 arboxymethyl-lysine (CML) and
lysinoalanine (
LAL) contents.
116 dotoxin using the limulus amebocyte
lystate (
LAL) gel clot method.
117 Lentiviral-
mediated LAL knockdown in the 3T3L1 mouse cell line to mimic the
118 In this study, we found that
murine LAL exhibits RE hydrolase activity.
119 zygote knockout mice (lal -/lal-) produce
no LAL mRNA, protein or enzyme activity.
120 ctivity assays revealed the existence of
non-
LAL acid hydrolytic activities for TG and RE.
121 Interestingly, this
non-
LAL acid TG hydrolytic activity was elevated in lysosome
122 +/lal- mice have approximately 50% of
normal LAL activity and do not show lipid accumulation.
123 NCTC 8325 and its identification as a
novel LAL.
124 Through week 12
of LAL-CL04, these seven patients also showed mean decrease
125 The unparalleled recognition abilities
of LAL biosensors perched with remarkable sensitivity, high
126 acological inhibition or genetic ablation
of LAL in murine liver largely reduced in vitro acid RE hyd
127 Ablation
of LAL suppresses immune rejection and allows growth of hum
128 urring in the tested sample upon addition
of LAL.
129 Western blot analysis
of LAL embryos showed an increase in both total and polymer
130 e started from the bibliographic analysis
of LAL synthesis of Cu-based nanocrystals to identify the r
131 The coagulation
of LAL is commonly used to signal the presence of endotoxin
132 To understand that the expression
of LAL mRNA and protein is tissue and cell specifically reg
133 Pharmacological inhibition
of LAL in the human hepatocyte cell line HepG2, incubated w
134 mides, esters, and ketones for inhibition
of LAL.
135 azole carbamates are effective inhibitors
of LAL.
136 oduced system are more than 90% reduction
of LAL consumption, from 100 uL/reaction to 9.6 uL/reaction
137 To dissect the functional role
of LAL for neutral lipid ester mobilization in the liver, w
138 To test the functional role
of LAL in hepatocyte, hepatocyte-specific expression of hum
139 ice provide a model to determine the role
of LAL in lipid metabolism and the pathogenesis of its defi
140 also contain retinyl esters (REs), a role
of LAL in the clearance of endocytosed REs has not been rep
141 These results indicate a crucial role
of LAL-regulated mTOR signaling in the production and funct
142 fied a second population, including a
single LAL neuron pair, that bidirectionally regulates ground s
143 Conversely, mice with adipose-
specific LAL overexpression (Adpn-rtTA x TetO-hLAL) gained less w
144 The adipose-
specific LAL-overexpressing mouse phenotype depends on the housin
145 The adipose-
specific LAL-overexpressing mouse phenotype depends on the housin
146 only approximately 3%-5% of normally
spliced LAL.
147 e eligible to enroll in the extension
study (
LAL-CL04) in which they again received four once-weekly
148 with a history of laser refractive
surgery,
LAL implantation and postimplantation adjustment provide
149 These results support a concept
that LAL in hepatocytes is a critical metabolic enzyme in con
150 These results support a concept
that LAL is a critical metabolic enzyme in lung epithelial ce
151 These observations conclude
that LAL-regulated lipid metabolism is essential to maintain
152 These studies demonstrate
that LAL in myeloid cells plays a critical role in maintainin
153 These results provide evidence
that LAL is an important regulator of myelopoiesis during hem
154 Our results indicate
that LAL has a critical role in regulating MDSCs' ability to
155 In summary, our results indicate
that LAL is the major acid RE hydrolase and required for func
156 Our results indicate
that LAL regulates EC functions through interaction with MDSC
157 n livers from hep-LAL-ko mice indicates
that LAL is limiting for CE turnover, but not for TG and RE t
158 dial circulating RE content, indicating
that LAL is required for efficient nutritional vitamin A avai
159 Our data suggest
that LAL/Lipa is involved in the degradation of a specific pr
160 The LAL endotoxin detection limit for samples dispersed in C
161 liposomes, but did not appear to affect
the LAL assay sensitivity once the liposomes were completely
162 Although
the LAL-generated Cu nanocrystals may be present in a range
163 t not DSPG, at 10 mol% further decreased
the LAL endotoxin detection limit.
164 Mice double homozygous for
the LAL and MMR deficiences (lal-/-;MMR-/-) showed phLAL upt
165 In
the LAL-deficient (lal(-/-)) mouse model, melanoma metastasi
166 L to consume unpolymerized macromeres in
the LAL.
167 ultraviolet light to alter the shape of
the LAL and hence its refractive power.
168 solubilization and/or the sensitivity of
the LAL assay.
169 A targeted disruption of
the LAL locus produced a null (lal( -/-)) mouse model that m
170 e equation giving the best prediction of
the LAL results.
171 tor neurons in the caudal muscle band of
the LAL.
172 ents, we applied 2 lock-in treatments to
the LAL to consume unpolymerized macromeres in the LAL.
173 ation in HSCs during activation in vitro
The LAL inhibitor increased the levels of TAG, cholesteryl e
174 l refractive surgery were implanted with
the LAL during cataract surgery performed at a single clinic
175 Therefore,
LAL, its downstream genes, and lipid mediators all play
176 ral TVA muscle compared with the fast-
twitch LAL and lumbrical muscles.
177 ole library of nanomaterials available
using LAL, has been until now an empirical endeavor based on c
178 re HPS function relative to controls
whereas LAL was associated with delayed conversion to apical ini
179 or LAL inhibition by these compounds
whereby LAL transiently carbamoylates the enzyme similarly to pr
180 LPS were quantified
with LAL assay (KQCL test).
181 aching significance (P=0.061) were seen
with LAL only.