(Q53661449)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

25 February 2020

title

Steatogenesis in adult-onset type II citrullinemia is associated with down-regulation of PPARα (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

25 February 2020

main subject

citrullinemia type II

1 reference

1 reference

12

Eiji Tanaka

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

25 February 2020

author name string

Michiharu Komatsu

1

1 reference

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25 February 2020

Takefumi Kimura

2

1 reference

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25 February 2020

Masahide Yazaki

3

1 reference

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25 February 2020

Naoki Tanaka

4

1 reference

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25 February 2020

Yang Yang

5

1 reference

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25 February 2020

Takero Nakajima

6

1 reference

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25 February 2020

Akira Horiuchi

7

1 reference

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25 February 2020

Zhong-Ze Fang

8

1 reference

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25 February 2020

Satoru Joshita

9

1 reference

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25 February 2020

Akihiro Matsumoto

10

1 reference

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25 February 2020

Takeji Umemura

11

1 reference

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25 February 2020

Frank J Gonzalez

13

1 reference

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25 February 2020

Shu-Ichi Ikeda

14

1 reference

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25 February 2020

Toshifumi Aoyama

15

1 reference

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25 February 2020

publication date

20 December 2014

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

Biochimica et Biophysica Acta

25 February 2020

published in

1 reference

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25 February 2020

volume

1852

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25 February 2020

issue

3

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25 February 2020

page(s)

473-481

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

Peroxisome proliferator-activated receptor alpha mediates the adaptive response to fasting

25 February 2020

cites work

1 reference

12 December 2020

The gene mutated in adult-onset type II citrullinaemia encodes a putative mitochondrial carrier protein

1 reference

12 December 2020

Human fatty acid omega-hydroxylase, CYP4A11: determination of complete genomic sequence and characterization of purified recombinant protein

1 reference

12 December 2020

Identification of two novel mutations in the SLC25A13 gene and detection of seven mutations in 102 patients with adult-onset type II citrullinemia

1 reference

12 December 2020

The peroxisome proliferator-activated receptor alpha regulates amino acid metabolism.

1 reference

12 December 2020

Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria

1 reference

12 December 2020

Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2−ΔΔCT Method

1 reference

12 December 2020

Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in mice.

1 reference

12 December 2020

Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency

1 reference

12 December 2020

The mitochondrial transporter family (SLC25): physiological and pathological implications

1 reference

12 December 2020

Hepatocellular Carcinoma in a Case of Adult-onset Type II Citrullinemia

1 reference

12 December 2020

Peroxisome proliferator-activated receptor alpha target genes

1 reference

12 December 2020

Feasibility of auxiliary partial orthotopic liver transplantation from living donors for patients with adult-onset type II citrullinemia

1 reference

12 December 2020

Peroxisome proliferator-activated receptor alpha protects against alcohol-induced liver damage

1 reference

12 December 2020

Abnormal expression of urea cycle enzyme genes in juvenile visceral steatosis (jvs) mice

1 reference

12 December 2020

Chronic pancreatitis associated with adult-onset type II citrullinemia: clinical and pathologic findings

1 reference

12 December 2020

Risk of worsened encephalopathy after intravenous glycerol therapy in patients with adult-onset type II citrullinemia (CTLN2).

1 reference

12 December 2020

Design and validation of a histological scoring system for nonalcoholic fatty liver disease

1 reference

12 December 2020

Frequency and distribution in East Asia of 12 mutations identified in the SLC25A13 gene of Japanese patients with citrin deficiency

1 reference

12 December 2020

Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier

1 reference

12 December 2020

Free fatty acids induce JNK-dependent hepatocyte lipoapoptosis

1 reference

12 December 2020

A lean man with nonalcoholic fatty liver disease

1 reference

12 December 2020

Adult-onset type II citrullinemia associated with idopathic hypertriglyceridemia as a preceding feature

1 reference

12 December 2020

Underlying mechanisms of pharmacology and toxicity of a novel PPAR agonist revealed using rodent and canine hepatocytes.

1 reference

12 December 2020

PPARalpha activation is essential for HCV core protein-induced hepatic steatosis and hepatocellular carcinoma in mice

1 reference

12 December 2020

Highly purified eicosapentaenoic acid treatment improves nonalcoholic steatohepatitis

1 reference

12 December 2020

Primary liver carcinoma exhibiting dual hepatocellular-biliary epithelial differentiations associated with citrin deficiency: a case report

1 reference

12 December 2020

Identification of 13 novel mutations including a retrotransposal insertion in SLC25A13 gene and frequency of 30 mutations found in patients with citrin deficiency

1 reference

12 December 2020

Citrin deficiency as a cause of chronic liver disorder mimicking non-alcoholic fatty liver disease

1 reference

12 December 2020

Citrin deficiency, a perplexing global disorder.

1 reference

12 December 2020

Long-chain fatty acid effects on peroxisome proliferator-activated receptor-alpha-regulated genes in Madin-Darby bovine kidney cells: optimization of culture conditions using palmitate

1 reference

12 December 2020

Conventional diet therapy for hyperammonemia is risky in the treatment of hepatic encephalopathy associated with citrin deficiency

1 reference

12 December 2020

Down-regulation of SREBP-1c is associated with the development of burned-out NASH.

1 reference

12 December 2020

Eicosapentaenoic acid improves hepatic steatosis independent of PPARα activation through inhibition of SREBP-1 maturation in mice

1 reference

12 December 2020

Who pulls the trigger: JNK activation in liver lipotoxicity?

1 reference

12 December 2020

Microvesicular steatosis of the liver

1 reference

12 December 2020

Inhibition of c-Jun-N-terminal kinase increases cardiac peroxisome proliferator-activated receptor alpha expression and fatty acid oxidation and prevents lipopolysaccharide-induced heart dysfunction.

1 reference

12 December 2020

The characteristics of food intake in patients with type II citrullinemia

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12 December 2020

A new Caucasian case of neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD): a clinical, molecular, and functional study

1 reference

12 December 2020

Disruption of phospholipid and bile acid homeostasis in mice with nonalcoholic steatohepatitis.

1 reference

12 December 2020

Clinical characteristics of young-onset ischemic colitis.

1 reference

12 December 2020

Hepatic Cerebroside Sulfotransferase Is Induced by PPARα Activation in Mice

1 reference

12 December 2020

The human liver fatty acid binding protein (FABP1) gene is activated by FOXA1 and PPARα; and repressed by C/EBPα: Implications in FABP1 down-regulation in nonalcoholic fatty liver disease

1 reference

12 December 2020

SLC25A13 gene analysis in citrin deficiency: sixteen novel mutations in East Asian patients, and the mutation distribution in a large pediatric cohort in China

1 reference

12 December 2020

Transcriptional Regulation of the Mitochondrial Citrate and Carnitine/Acylcarnitine Transporters: Two Genes Involved in Fatty Acid Biosynthesis and β-oxidation

1 reference

12 December 2020

Cytochrome P-450 hPCN3, a novel cytochrome P-450 IIIA gene product that is differentially expressed in adult human liver. cDNA and deduced amino acid sequence and distinct specificities of cDNA-expressed hPCN1 and hPCN3 for the metabolism of [...]

1 reference

12 December 2020

Molecular analysis of argininosuccinate synthetase deficiency in human fibroblasts

1 reference

12 December 2020

Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients

1 reference

12 December 2020

Focal glycogenosis of the liver in disorders of ureagenesis: its occurrence and diagnostic significance

1 reference

12 December 2020

Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor alpha (PPARalpha)

1 reference

12 December 2020

10.1016/J.BBADIS.2014.12.011

Identifiers

DOI

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25533124%20AND%20SRC:MED&resulttype=core&format=json

25 February 2020

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25 February 2020

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