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内部出版物
Wiley电子期刊(2)
SpringerLink电子期刊(138)
Elsevier电子期刊(300)
Springer电子图书(1)
NATURE电子期刊(2)
ACS电子期刊(8)
在“
Elsevier电子期刊
”中,
命中:
300
条,耗时:0.1099439 秒
在所有数据库中总计命中:
451
条
1.
Efficacy of bezafibrate on fibroblasts of glutaric
acid
emia type II patients evaluated using an in vitro probe acylcarnitine assay
作者:
Kenji Yamada
a
;
k-yamada@med.shimane-u.ac.jp
;
Hironori Kobayashi
a
;
Ryosuke Bo
a
;
b
;
Jamiyan Purevsuren
a
;
Yuichi Mushimoto
a
;
Tomoo Takahashi
a
;
Yuki Hasegawa
a
;
Takeshi Taketani
a
;
Seiji Fukuda
a
;
Seiji Yamaguchi
a
关键词:
Bezafibrate
;
Glutaric
acid
emia type II
;
Multiple acyl-CoA dehydrogenase deficiency
;
In vitro probe assay
;
Fatty
acid
oxidation
disorders
刊名:Brain and Development
出版年:2017
2.
The molecular structure of thio-ether
fatty
acid
s influences PPAR-dependent regulation of lipid metabolism
作者:
Jenny Lund
a
;
&dagger
;
;
Camilla Stensrud
a
;
&dagger
;
;
Rajender
b
;
c
;
Pavol Bohov
d
;
G. Hege Thoresen
a
;
e
;
Rolf K. Berge
d
;
f
;
Michael Wright
b
;
g
;
Ahmed Kamal
c
;
Arild C. Rustan
a
;
Andrew D. Miller
c
;
g
;
h
;
Jon Skorve
d
;
jon.skorve@k2.uib.no" class="auth_mail" title="E-mail the corresponding author
关键词:
THEFA
;
thio-ether
fatty
acid
;
FA
;
fatty
acid
;
MUFA
;
monounsaturated
fatty
acid
;
SFA
;
saturated
fatty
acid
;
TTA
;
2-(tetradecylthio)acetic
acid
;
TTA-PC
;
1
;
2-di-[2-(tetradecylthio)acetyl]-sn-glycero-3-phosphocholine
;
TTA-MAG
;
1-[2-(tetradecylthio)acetyl]-sn-glycerol
;
dTTA
;
2-(tetradec-[7-enyl]thio)acetic
acid
;
dTTA-PC
;
1
;
2-di-[2-(tetradec-[7-enyl]thio)acetyl]-sn-glycero-3-phosphocholine
;
lyso-dTTA-PC
;
1-[2-(tetradec-[7-enyl]thio)acetyl]-sn-glycero-3-phosphocholine
;
dTTA-MAG
;
1-[2-(tetradec-[7-enyl]
刊名:Bioorganic & Medicinal Chemistry
出版年:2016
3.
Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-
fatty
acid
oxidation
disorders
作者:
J. Vockley
a
;
vockleyg@upmc.edu" class="auth_mail" title="E-mail the corresponding author
;
J. Charrow
b
;
J. Ganesh
c
;
M. Eswara
d
;
G.A. Diaz
e
;
E. McCracken
a
;
R. Conway
f
;
G.M. Enns
g
;
J. Starr
h
;
R. Wang
h
;
J.E. Abdenur
h
;
J. Sanchez-de-Toledo
a
;
D.L. Marsden
i
关键词:
Cardiomyopathy
;
Metabolic
disorders
;
Fatty
acid
oxidation
disorders
;
Triheptanoin
刊名:Molecular Genetics and Metabolism
出版年:2016
4.
Clinical, biochemical and molecular investigation of adult-onset glutaric
acid
emia type II: Characteristics in comparison with pediatric cases
作者:
Kenji Yamada
a
;
k-yamada@med.shimane-u.ac.jp" class="auth_mail" title="E-mail the corresponding author
;
Hironori Kobayashi
a
;
Ryosuke Bo
a
;
Tomoo Takahashi
a
;
Jamiyan Purevsuren
a
;
Yuki Hasegawa
a
;
Takeshi Taketani
a
;
Seiji Fukuda
a
;
Takuya Ohkubo
b
;
Takanori Yokota
b
;
Mutsufusa Watanabe
c
;
Taiji Tsunemi
d
;
Hidehiro Mizusawa
e
;
Hiroshi Takuma
f
;
Ayako Shioya
f
;
Akiko Ishii
f
;
Akira Tamaoka
f
;
Yosuke Shigematsu
g
;
Hideo Sugie
h
;
Seiji Yamaguchi
a
关键词:
Multiple acyl-CoA dehydrogenase deficiency (glutaric
acid
emia type II)
;
Adult onset
;
Myopathy
;
Serum acylcarnitine
;
Immunoblotting
;
In vitro probe acylcarnitine assay
刊名:Brain and Development
出版年:2016
5.
Carnitine palmitoyltransferase 1C: From cognition to cancer
作者:
Nú
;
ria Casals
a
;
b
;
ncasals@uic.es" class="auth_mail" title="E-mail the corresponding author
;
Victor Zammit
c
;
V.A.Zammit@warwick.ac.uk" class="auth_mail" title="E-mail the corresponding author
;
Laura Herrero
a
;
d
;
Rut Fadó
;
a
;
b
;
Rosalí
;
a Rodrí
;
guez-Rodrí
;
guez
a
;
b
;
Dolors Serra
a
;
d
关键词:
ACC
;
acetyl-CoA carboxylase
;
ACO
;
aconitase
;
AICAR
;
5-aminoimidazole-4-1-b-d-ribofuranoside
;
AMPK
;
AMP-dependent protein kinase
;
Arc
;
Arcuate
;
BSX
;
brain-specific homeobox
;
cAMP
;
cyclic AMP
;
CNS
;
central nervous system
;
COT
;
carnitine octanoyltransferase
;
CPT
;
carnitine palmitoyltransferase
;
CrAT
;
carnitine acetyltransferase
;
CREB
;
cAMP response element-binding protein
;
DG
;
deoxyglucose
;
ER
;
endoplasmic reticulum
;
ERRα
;
estrogen-related receptor α
;
FA
;
fatty
acid
;
FAO
;
fatty
acid
oxidation
;
FAS
刊名:Progress in Lipid Research
出版年:2016
6.
Altered heart proteome in fructose-fed Fisher 344 rats exposed to bisphenol A
作者:
S.A. Ljunggren
a
;
Stefan.ljunggren@liu.se" class="auth_mail" title="E-mail the corresponding author
;
M. Iggland
b
;
Madeleine.iggland@liu.se" class="auth_mail" title="E-mail the corresponding author
;
M. Rö
;
nn
c
;
Monika.ronn@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author
;
L. Lind
d
;
Lars.lind@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author
;
P.M. Lind
c
;
Monica.lind@medsci.uu.se" class="auth_mail" title="E-mail the corresponding author
;
H. Karlsson
a
;
helen.m.karlsson@liu.se" class="auth_mail" title="E-mail the corresponding author
关键词:
2DE
;
two-dimensional gel electrophoresis
;
BPA
;
Bisphenol A
;
FA
;
Formic
acid
;
FDR
;
False discovery rate
;
DHB
;
2
;
5-dihydroxybenzoic
acid
;
MALDI-TOF
;
matrix associated laser desorption/ionization time-of-flight
;
MS
;
mass spectrometry
;
MS/MS
;
tandem mass spectrometry
;
nLC
;
nano-liquid chromatography
;
S1P
;
sphingosine-1-phosphate
;
TFA
;
Trifluoroacetic
acid
刊名:Toxicology
出版年:2016
7.
Peripheral neuropathy in patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency - A follow-up EMG study of 12 patients
作者:
Immonen Tuuli
a
;
tuuli.immonen@hus.fi" class="auth_mail" title="E-mail the corresponding author
;
Ahola Emilia
a
;
Toppila Jussi
b
;
Lapatto Risto
a
;
Tyni Tiina
a
;
Lauronen Leena
b
关键词:
Fatty
acid
oxidation
disorders
;
Inborn errors of metabolism
;
Mitochondrial
fatty
acid
β-
oxidation
;
Polyneuropathy
刊名:European Journal of Paediatric Neurology
出版年:2016
8.
Long-term major clinical outcomes in patients with long chain
fatty
acid
oxidation
disorders
before and after transition to triheptanoin treatment鈥擜 retrospective chart review
作者:
Jerry Vockley
a
;
b
;
gerard.vockley@chp.edu" class="auth_mail" title="E-mail the corresponding author
;
Deborah Marsden
c
;
Elizabeth McCracken
a
;
Stephanie DeWard
a
;
Amanda Barone
a
;
Kristen Hsu
c
;
Emil Kakkis
c
关键词:
CACT
;
carnitine acylcarnitine translocase
;
CM
;
cardiomyopathy
;
CRF
;
case report forms
;
ECHO
;
echocardiography
;
ER
;
emergency room
;
LC-FAOD
;
long chain
fatty
acid
oxidation
disorder
;
MCT
;
Medium Chain Triglyceride
;
TFP
;
trifunctional protein
;
CK
;
creatine kinase
;
CPT
;
carnitine palmitoyltransferase deficiencies
;
ICU
;
intensive care unit
;
IQR
;
interquartile range
;
IV
;
intravenous
;
LCHAD
;
long-chain 3-hydroxy-acyl-CoA dehydrogenase
;
VLCAD
;
very long chain acyl-CoA dehydrogenase
刊名:Molecular Genetics and Metabolism
出版年:2015
9.
Doubling diet fat on sugar ratio in children with mitochondrial OXPHOS
disorders
: Effects of a randomized trial on resting energy expenditure, diet induced thermogenesis and body composition
作者:
Laurent Bé
;
ghin
a
;
b
;
laurent.beghin@chru-lille.fr" class="auth_mail" title="E-mail the corresponding author
;
Sté
;
phanie Coopman
a
;
Stephanie.coopman@chru-lille.fr" class="auth_mail" title="E-mail the corresponding author
;
Manuel Schiff
c
;
manuel.schiff@aphp.fr" class="auth_mail" title="E-mail the corresponding author
;
Joseph Vamecq
d
;
joseph.vamecq@inserm.fr" class="auth_mail" title="E-mail the corresponding author
;
Karine Mention-Mulliez
e
;
karine.mention@chru-lille.fr" class="auth_mail" title="E-mail the corresponding author
;
Ré
;
gis Hankard
f
;
regis.hankard@univ-tours.fr" class="auth_mail" title="E-mail the corresponding author
;
Jean-Marie Cuisset
g
;
Hé
;
lè
;
ne Ogier
c
;
Fré
;
dé
;
ric Gottrand
a
;
b
;
Frederic.gottrand@chru-lille.fr" class="auth_mail" title="E-mail the corresponding author
;
Dries Dobbelaere
e
;
dries.dobbelaere@chru-lille.fr" class="auth_mail" title="E-mail the corresponding author
关键词:
Mitochondrial
disorders
or diseases
;
Energy expenditure
;
Diet induced thermogenesis
;
Body composition
刊名:Clinical Nutrition
出版年:2016
10.
The modern interleukin-1 superfamily: Divergent roles in obesity
作者:
Man K.S. Lee
a
;
b
;
Laurent Yvan-Charvet
c
;
Seth L. Masters
d
;
Andrew J. Murphy
a
;
b
;
andrew.murphy@bakeridi.edu.au
关键词:
IL-1β
;
IL-18
;
IL-33
;
Obesity
;
Cytokines
;
Fatty
acid
oxidation
刊名:Seminars in Immunology
出版年:2016
1
2
3
4
5
6
7
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