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AGC2 (Citrin) Deficiency-From Recognition of the Disease till Construction of Therapeutic Procedures

Biomolecules (Basel, Switzerland), 2020-07, Vol.10 (8), p.1100

2020

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Reduced N-Acetylaspartate Levels in Mice Lacking Aralar, a Brain- and Muscle-type Mitochondrial Aspartate-glutamate Carrier

The Journal of biological chemistry, 2005-09, Vol.280 (35), p.31333-31339

2005

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AGC1‐malate aspartate shuttle activity is critical for dopamine handling in the nigrostriatal pathway

Journal of neurochemistry, 2013-02, Vol.124 (3), p.347-362

2013

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Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse: Urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency

Biochimica et biophysica acta. Molecular basis of disease, 2015-09, Vol.1852 (9), p.1787-1795

2015

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Citrin/Mitochondrial Glycerol-3-phosphate Dehydrogenase Double Knock-out Mice Recapitulate Features of Human Citrin Deficiency

The Journal of biological chemistry, 2007-08, Vol.282 (34), p.25041-25052

2007

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Slc25a13-Knockout Mice Harbor Metabolic Deficits but Fail To Display Hallmarks of Adult-Onset Type II Citrullinemia

Molecular and Cellular Biology, 2004-01, Vol.24 (2), p.527-536

2004

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Failure of the feeding response to fasting in carnitine-deficient juvenile visceral steatosis (JVS) mice: Involvement of defective acyl-ghrelin secretion and enhanced corticotropin-releasing factor signaling in the hypothalamus

Biochimica et biophysica acta, 2009-11, Vol.1792 (11), p.1087-1093

2009

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Attenuation of Cardiac Hypertrophy in Carnitine-Deficient Juvenile Visceral Steatosis (JVS) Mice Achieved by Lowering Dietary Lipid

Journal of biochemistry (Tokyo), 2006-02, Vol.139 (2), p.263-270

2006

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Ca²⁺ Activation Kinetics of the Two Aspartate-Glutamate Mitochondrial Carriers, Aralar and Citrin: ROLE IN THE HEART MALATE-ASPARTATE NADH SHUTTLE

The Journal of biological chemistry, 2007-03, Vol.282 (10), p.7098-7106

2007

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Essential role of aralar in the transduction of small Ca2+ signals to neuronal mitochondria

The Journal of biological chemistry, 2006-01, Vol.281 (2), p.1039

2006

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Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action

FEBS letters, 1996-12, Vol.399 (3), p.310-312

1996

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Secondary abnormality of carnitine biosynthesis results from carnitine reabsorptional system defect in juvenile visceral steatosis mice

Biochimica et biophysica acta, 1997-12, Vol.1362 (2), p.263-268

1997

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Pancreatic secretory trypsin inhibitor gene is highly expressed in the liver of adult-onset type II citrullinemia

FEBS letters, 1995-09, Vol.372 (1), p.69-73

1995

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Pyruvate dehydrogenase kinase 4 mRNA is increased in the hypertrophied ventricles of carnitine-deficient juvenile visceral steatosis (JVS) mice

BioFactors (Oxford), 1999, Vol.10 (2-3), p.301-309

1999

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Molecular Cloning and Expression of Serum Calcium- decreasing Factor (Caldecrin) (∗)

The Journal of biological chemistry, 1995-12, Vol.270 (51), p.30315-30321

1995

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Molecular cloning and expression of human caldecrin

FEBS letters, 1996-05, Vol.386 (1), p.26-28

1996

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Suppressed Expression of the Urea Cycle Enzyme Genes in the Liver of Carnitine-Deficient Juvenile Visceral Steatosis (JVS) Mice in Infancy and during Starvation in Adulthood

Journal of biochemistry (Tokyo), 1997-01, Vol.121 (1), p.172-177

1997

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Caldecrin Is a Novel-Type Serine Protease Expressed in Pancreas, but Its Homologue, Elastase IV, Is an Artifact during Cloning Derived from Caldecrin Gene

Journal of biochemistry (Tokyo), 1998-03, Vol.123 (3), p.546-554

1998

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Prolonged effect of single carnitine administration on fasted carnitine-deficient JVS mice regarding their locomotor activity and energy expenditure

Biochimica et biophysica acta, 2006-10, Vol.1761 (10), p.1191-1199

2006

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

Reviews of physiology, biochemistry and pharmacology, 1987-01, Vol.108, p.21

1987

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Intracellular Distribution and Biosynthesis of Lipoamide Dehydrogenase in Rat Liver

Journal of biochemistry (Tokyo), 1982-01, Vol.91 (2), p.553-561

1982

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Amino Acid Sequence of Rat Argininosuccinate Lyase Deduced from cDNA

Journal of biochemistry (Tokyo), 1988-01, Vol.103 (1), p.177-181

1988

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Molecular basis of citrin deficiency

Seikagaku. The Journal of Japanese Biochemical Society, 2004-12, Vol.76 (12), p.1543

2004

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

Advances in enzyme regulation, 2001-01, Vol.41 (1), p.325-342

2001

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Regulation of urea synthesis in rat liver: Inhibition of urea synthesis by L-norvaline

Journal of biochemistry (Tokyo), 1979-01, Vol.86 (3), p.745-750

1979

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AGC2 (Citrin) Deficiency-From Recognition of the Disease till Construction of Therapeutic Procedures

Reduced N-Acetylaspartate Levels in Mice Lacking Aralar, a Brain- and Muscle-type Mitochondrial Aspartate-glutamate Carrier

AGC1‐malate aspartate shuttle activity is critical for dopamine handling in the nigrostriatal pathway

Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse: Urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency

Citrin/Mitochondrial Glycerol-3-phosphate Dehydrogenase Double Knock-out Mice Recapitulate Features of Human Citrin Deficiency

Slc25a13-Knockout Mice Harbor Metabolic Deficits but Fail To Display Hallmarks of Adult-Onset Type II Citrullinemia

Failure of the feeding response to fasting in carnitine-deficient juvenile visceral steatosis (JVS) mice: Involvement of defective acyl-ghrelin secretion and enhanced corticotropin-releasing factor signaling in the hypothalamus

Attenuation of Cardiac Hypertrophy in Carnitine-Deficient Juvenile Visceral Steatosis (JVS) Mice Achieved by Lowering Dietary Lipid

Ca²⁺ Activation Kinetics of the Two Aspartate-Glutamate Mitochondrial Carriers, Aralar and Citrin: ROLE IN THE HEART MALATE-ASPARTATE NADH SHUTTLE

Essential role of aralar in the transduction of small Ca2+ signals to neuronal mitochondria

Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action

Secondary abnormality of carnitine biosynthesis results from carnitine reabsorptional system defect in juvenile visceral steatosis mice

Pancreatic secretory trypsin inhibitor gene is highly expressed in the liver of adult-onset type II citrullinemia

Pyruvate dehydrogenase kinase 4 mRNA is increased in the hypertrophied ventricles of carnitine-deficient juvenile visceral steatosis (JVS) mice

Molecular Cloning and Expression of Serum Calcium- decreasing Factor (Caldecrin) (∗)

Molecular cloning and expression of human caldecrin

Suppressed Expression of the Urea Cycle Enzyme Genes in the Liver of Carnitine-Deficient Juvenile Visceral Steatosis (JVS) Mice in Infancy and during Starvation in Adulthood

Caldecrin Is a Novel-Type Serine Protease Expressed in Pancreas, but Its Homologue, Elastase IV, Is an Artifact during Cloning Derived from Caldecrin Gene

Prolonged effect of single carnitine administration on fasted carnitine-deficient JVS mice regarding their locomotor activity and energy expenditure

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

Intracellular Distribution and Biosynthesis of Lipoamide Dehydrogenase in Rat Liver

Amino Acid Sequence of Rat Argininosuccinate Lyase Deduced from cDNA

Molecular basis of citrin deficiency

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

Regulation of urea synthesis in rat liver: Inhibition of urea synthesis by L-norvaline

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