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

Md. Abdul Jalil, Laila Begum, Laura Contreras, +15

Journal of Biological Chemistry

Cellular PhysiologySocial SciencesOxidative StressAlzheimer's DiseaseMyelin Lipid Precursor +20

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

Tomotsugu Yasuda, Naoki Yamaguchi, Keiko Kobayashi, +8

Human Genetics

Mendelian DisorderGenetic DisorderGeneticsInherited Metabolic DiseaseNovel Mutations +6

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

Yao Lu, Keiko Kobayashi, Miharu Ushikai, +13

Journal of Human Genetics

Genetic DisorderGeneticsHuman PolymorphismPathologySlc25a13 Gene +7

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

Takeyori Saheki, Mikio Iijima, Meng Xian Li, +15

Journal of Biological Chemistry

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

David S. Sinasac, Mitsuaki Moriyama, Md. Abdul Jalil, +8

Molecular and Cellular Biology

Metabolic DisorderGeneticsImmunologyMetabolic RemodelingOxidative Stress +20

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

Laila Begum, Md. Abdul Jalil, Keiko Kobayashi, +7

Biochimica et Biophysica Acta (BBA) - Gene Structure and Expression

Urea CycleMolecular PhysiologyMitochondrial BiogenesisBiochemistryMitochondrial Function +10

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

Mikio Iijima, Abdul Jalil, Laila Begum, +7

Advances in Enzyme Regulation

Mitochondrial BiogenesisMitochondrial FunctionPhysiologySubcellular LocalizationPathology +4

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

Mitsuaki Moriyama, Meng Xian Li, Keiko Kobayashi, +8

Journal of Hepatology

UrologyPhysiologyMetabolismMedicineOxidative Stress +1

Fasting-induced reduction in locomotor activity and reduced response of orexin neurons in carnitine-deficient mice

Goichiro Yoshida, Meng Xian Li, Masahisa Horiuchi, +14

Neuroscience Research

Synaptic PlasticityOrexin NeuronsNeurophysiologySynaptic TransmissionPhysiology +8

Aberrations of ammonia metabolism in ornithine carbamoyltransferase-deficient spf-ash mice and their prevention by treatment with urea cycle intermediate amino acids and an ornithine aminotransferase inactivator

Meng Xian Li, Toshihiro Nakajima, Tomoko Fukushige, +3

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease

BiochemistryReactive Nitrogen SpecieAmmonia MetabolismOrnithine Aminotransferase InactivatorToxicology +6