Reduced carbohydrate intake in citrin-deficient subjects (Q46642310)

23 December 2017

title

Reduced carbohydrate intake in citrin-deficient subjects (English)

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23 December 2017

author name string

T Saheki

1

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23 December 2017

K Kobayashi

2

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23 December 2017

M Terashi

3

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23 December 2017

T Ohura

4

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23 December 2017

Y Yanagawa

5

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23 December 2017

Y Okano

6

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23 December 2017

T Hattori

7

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23 December 2017

H Fujimoto

8

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23 December 2017

K Mutoh

9

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Z Kizaki

10

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A Inui

11

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23 December 2017

publication date

14 April 2008

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23 December 2017

Journal of Inherited Metabolic Disease

published in

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23 December 2017

volume

31

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23 December 2017

issue

3

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23 December 2017

page(s)

386-394

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23 December 2017

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

cites work

1 reference

Crossref

https://api.crossref.org/works/10.1007%2FS10545-008-0752-X

Palmitate inhibits liver glycolysis. Involvement of fructose 2,6-bisphosphate in the glucose/fatty acid cycle

21 January 2018

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Crossref

https://api.crossref.org/works/10.1007%2FS10545-008-0752-X

Hepatocellular carcinoma associated with adult-type citrullinemia

21 January 2018

1 reference

12 December 2020

Effectiveness of carbohydrate-restricted diet and arginine granules therapy for adult-onset type II citrullinemia: a case report of siblings showing homozygous SLC25A13 mutation with and without the disease

1 reference

12 December 2020

Infantile cholestatic jaundice associated with adult-onset type II citrullinemia

1 reference

12 December 2020

Oral arginine does not stimulate an increase in insulin concentration but delays glucose disposal

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12 December 2020

Leucine promotes glucose uptake in skeletal muscles of rats

1 reference

12 December 2020

Pathogenesis of adult-onset type II citrullinemia caused by deficiency of citrin, a mitochondrial solute carrier protein: tissue and subcellular localization of citrin

1 reference

12 December 2020

Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients

1 reference

12 December 2020

A mechanism for fatty acid inhibition of glucose utilization in liver. Role of xylulose 5-P

1 reference

12 December 2020

Slc25a13-knockout mice harbor metabolic deficits but fail to display hallmarks of adult-onset type II citrullinemia

1 reference

12 December 2020

Pyruvate ameliorates the defect in ureogenesis from ammonia in citrin-deficient mice

1 reference

12 December 2020

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

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12 December 2020

Citrin/mitochondrial glycerol-3-phosphate dehydrogenase double knock-out mice recapitulate features of human citrin deficiency

1 reference

12 December 2020

Neonatal intrahepatic cholestasis caused by citrin deficiency: severe hepatic dysfunction in an infant requiring liver transplantation

1 reference

12 December 2020

Hyperdibasicaminoaciduria, hyperammonemia, and growth retardation: Treatment with arginine, lysine, and citrulline

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

Differential effect of protein and fat ingestion on blood glucose responses to high- and low-glycemic-index carbohydrates in noninsulin-dependent diabetic subjects.

1 reference

12 December 2020

Partial N-acetylglutamate synthetase deficiency in a 13-year-old girl: diagnosis and response to treatment with N-carbamylglutamate

1 reference

12 December 2020

Adult-onset type II citrullinemia and idiopathic neonatal hepatitis caused by citrin deficiency: involvement of the aspartate glutamate carrier for urea synthesis and maintenance of the urea cycle

1 reference

12 December 2020

A Chinese adult onset type II citrullinaemia patient with 851del4/1638ins23 mutations in the SLC25A13 gene

1 reference

12 December 2020

Effects of citrin deficiency in the perinatal period: feasibility of newborn mass screening for citrin deficiency

1 reference

12 December 2020

Neonatal presentation of adult-onset type II citrullinemia

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12 December 2020

Novel diagnostic approach to citrin deficiency: analysis of citrin protein in lymphocytes

1 reference

12 December 2020

Reversibility of serum NH3 level in a case of sudden onset and rapidly progressive case of type 2 citrullinemia.

1 reference

12 December 2020

Screening of SLC25A13 mutations in early and late onset patients with citrin deficiency and in the Japanese population: Identification of two novel mutations and establishment of multiple DNA diagnosis methods for nine mutations

1 reference

12 December 2020

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

1 reference

12 December 2020

Citrin deficiency presenting with ketotic hypoglycaemia and hepatomegaly in childhood

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12 December 2020

Molecular basis of citrin deficiency

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12 December 2020

Citrin deficiency: a novel cause of failure to thrive that responds to a high-protein, low-carbohydrate diet

1 reference

12 December 2020

A case of adult-onset type II citrullinemia--deterioration of clinical course after infusion of hyperosmotic and high sugar solutions.

1 reference

12 December 2020

Increasing dietary leucine intake reduces diet-induced obesity and improves glucose and cholesterol metabolism in mice via multimechanisms

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12 December 2020

Isoleucine, a blood glucose-lowering amino acid, increases glucose uptake in rat skeletal muscle in the absence of increases in AMP-activated protein kinase activity

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12 December 2020

Development of enzymes of glycerol metabolism in human fetal liver

1 reference

12 December 2020

A novel inborn error of metabolism detected by elevated methionine and/or galactose in newborn screening: neonatal intrahepatic cholestasis caused by citrin deficiency

1 reference

12 December 2020

Type II (adult onset) citrullinaemia: clinical pictures and the therapeutic effect of liver transplantation

1 reference

12 December 2020

Sibling cases of chronic recurrent hepatocerebral disease with hypercitrullinemia

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12 December 2020

Kinetic analysis of argininosuccinate synthetase in a variant form of citrullinaemia.

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12 December 2020

Mitochondrial aspartate glutamate carrier (citrin) deficiency as the cause of adult-onset type II citrullinemia (CTLN2) and idiopathic neonatal hepatitis (NICCD)

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12 December 2020

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

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12 December 2020

The reduced energy intake of rats fed a high-protein low-carbohydrate diet explains the lower fat deposition, but macronutrient substitution accounts for the improved glycemic control.

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12 December 2020

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

1 reference

12 December 2020

Molecular cloning of Aralar, a new member of the mitochondrial carrier superfamily that binds calcium and is present in human muscle and brain

1 reference

12 December 2020

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

1 reference

12 December 2020

Hereditary disorders of the urea cycle in man: biochemical and molecular approaches.

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12 December 2020

Diurnal fluctuation of blood ammonia levels in adult-type citrullinemia.

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12 December 2020

10.1007/S10545-008-0752-X

Identifiers

DOI

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23 December 2017

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23 December 2017