Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Volume 1852, Issue 3, March 2015, Pages 473-481
Steatogenesis in adult-onset type II citrullinemia is associated with down-regulation of PPARα
Under an Elsevier user license
open archive
Abbreviations
ACAA1
acetyl-CoA acyltransferase 1
ACACA
acetyl-CoA carboxylase α
ACACB
acetyl-CoA carboxylase β
ACADM
medium-chain acyl-CoA dehydrogenase
ACADVL
very-long-chain acyl-CoA dehydrogenase
ACLY
ATP citrate lyase
ACOX1
acyl-CoA oxidase 1
ACSL1
acyl-CoA synthetase long-chain family member 1
AGC
aspartate–glutamate carrier
ALT
alanine aminotransferase
APOB
apolipoprotein B
ASS
argininosuccinate synthase
AST
aspartate aminotransferase
BMI
body mass index
CAC
carnitine–acylcarnitine carrier
CAT
catalase
CD
citrin deficiency
CoA
coenzyme A
CPT1A
carnitine palmitoyl-CoA transferase 1α
CTLN2
adult-onset type II citrullinemia
CYBB
cytochrome b-245, β polypeptide
CYP4A11
cytochrome P450, family 4, subfamily A, polypeptide 11
DGAT
diacylglycerol O-acyltransferase
FA
fatty acid
FABP1
fatty acid-binding protein 1
FASN
fatty acid synthase
IL
interleukin
JNK
c-Jun-N-terminal kinase
LIPC
hepatic lipase
MDA
malondialdehyde
MTTP
microsomal triglyceride transfer protein
NAFLD
non-alcoholic fatty liver disease
NASH
non-alcoholic steatohepatitis
NF-κB
nuclear factor kappa B
NFKBIA
nuclear factor of kappa light polypeptide gene enhancer in B-cells inhibitor α
NICCD
neonatal intrahepatic cholestasis caused by citrin deficiency
NOX4
NADPH oxidase 4
PCR
polymerase chain reaction
p-JNK
phosphorylated JNK
PPAR
peroxisome proliferator-activated receptor
RXR
retinoid X receptor
SOD
superoxide dismutase
SREBF1
sterol regulatory element-binding transcription factor 1
TG
triglyceride
TGFB1
transforming growth factor β1
TNF
tumor necrosis factor α
US
ultrasonography
XDH
xanthine dehydrogenase
Keywords
Urea cycle disorder
SLC25A13
NAFLD
PPARα
Mitochondrial β-oxidation
JNK
Cited by (0)
- 1
These authors equally contributed to this study.
Copyright © 2014 Elsevier B.V. Published by Elsevier B.V. All rights reserved.