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Citrin is a mitochondrial aspartate-glutamate carrier primarily expressed in liver. Paper-13452760.
Moreover citrin and its isoform aralar were found to be aspartate glutamate carrier. Paper-9618273.
Pathogenesis and pathophysiology of citrin (a mitochondrial aspartate glutamate carrier) deficiency. Paper-9618273.
One case each of CPT-2 deficiency and citrin deficiency could not be detected in the newborn period. Paper-13424585.
Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier. Paper-12102732.
Citrin and aralar1 are Ca(2+)-stimulated aspartate/ glutamate transporters in mitochondria. Paper-9067706.
Citrin is a mitochondrial aspartate glutamate carrier primarily expressed in the liver, heart, and kidney. Paper-10420218.
Citrin knockdown also increased the expression of Bax and Bak, and reduced expression of Bcl-xL and Bcl-2. Paper-13452760.
Aralar1 and citrin were identified as calcium binding aspartate/glutamate carriers (AGC) in mitochondria. Paper-10211210.
Adult-onset type 2 citrullinaemia (CTLN2) is caused by a deficiency of the citrin protein encoded by the SLC25A13 gene. Paper-9639915.
Adult-onset citrullinemia (type II, CTLN2) has been attributed to citrin deficiency caused by mutations in the SLC25A13 gene. Paper-12245900.
Aralar1 and citrin are two isoforms of the mitochondrial aspartate/ glutamate carrier, one key constituent of the malate-aspartate NADH shuttle. Paper-10912623.
RESULTS: Regulator and Citrin K significantly reduced food intake in both experimental setups, while Super CitriMax HCA-600-SXS was less effective. Paper-11284809.
Homozygous SLC25A13 Mutation in a Taiwanese Patient with Adult-onset Citrullinemia Complicated with Steatosis and Hepatocellular Carcinoma. Paper-12245900.
Citrin and aralar1 are homologous proteins belonging to the mitochondrial carrier family with EF-hand Ca(2+)-binding motifs in their N-terminal domains. Paper-9067706.
The two mammalian AGCs, aralar and citrin, are members of the malate-aspartate NADH shuttle, and citrin, the liver AGC, is also a member of the urea cycle. Paper-12433553.
BACKGROUND/AIMS: Mutations in SLC25A13, encoding the mitochondrial aspartate-glutamate carrier citrin, cause adult-onset type II citrullinemia (CTLN2) in humans. Paper-11807450.
DNA analysis of the citrin gene and enzymatic assay of argininosuccinate synthetase in the liver led to a diagnosis of adult-onset type II citrullinemia (CTLN2). Paper-10101321.
Recently, we identified in man two AGC isoforms, aralar1 and citrin, which are regulated by calcium on the external face of the inner mitochondrial membrane. Paper-10129742.
Mitochondrial aspartate glutamate carrier ( citrin) deficiency as the cause of adult-onset type II citrullinemia (CTLN2) and idiopathic neonatal hepatitis (NICCD). Paper-9597441.
Adult-onset type II citrullinemia is caused by mutations in the SLC25A13 gene that encodes for citrin, and patients with this condition do not express citrin. Paper-13452760.
CONCLUSION: Regulator and Citrin K were shown to be potent inhibitors of food intake in rats, whereas Super CitriMax HCA-600-SXS showed only small and more inconsistent effects. Paper-11284809.
Adult-onset type II citrullinemia, characterized by a liver-specific argininosuccinate synthetase deficiency, is caused by a deficiency of citrin that is encoded by the SLC25A13 gene. Paper-8792370.
Adult-onset type II citrullinaemia, caused by deficiency of the citrin protein encoded by the SLC25A13 gene, is characterised by a liver-specific argininosuccinate synthetase deficiency. Paper-10016536.
Adult-onset type II citrullinemia (CTLN2) is characterized by a liver-specific argininosuccinate synthetase deficiency caused by a deficiency of the citrin protein encoded by the SLC25A13 gene. Paper-8708479.
These results demonstrate that citrin and aralar1 are isoforms of the hitherto unidentified aspartate/ glutamate carrier and explain why mutations in citrin cause type II citrullinemia in humans. Paper-9067706.
Although so far 14 CTLN2 patients with hepatocellular carcinoma have been reported, this report describes a unique case of liver carcinoma showing the features of both hepatocellular and cholangiocellular carcinoma. Paper-12938803.
Delivery may be a trigger for the development of CTLN2, while certain pathologic conditions associated with citrin gene abnormality are likely to induce hepatocellular carcinoma in patients with this disorder. Paper-10101321.
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. Paper-10420218.
Conversely, the cytosolic and the mitochondrial Ca2+ signals were the same in control cells and cells expressing the different aralar1 and citrin variants, thus ruling out an indirect effect through the Ca(2+)-sensitive dehydrogenases. Paper-10006375.
The activity of citrin and aralar1 as aspartate/ glutamate exchangers was stimulated by Ca(2+) on the external side of the inner mitochondrial membrane, where the Ca(2+)-binding domains of these proteins are localized. Paper-9067706.
CONCLUSIONS: Patients presenting with non-alcoholic fatty liver unrelated to obesity and metabolic syndrome might have citrin deficiency, and serum PSTI may be a useful indicator for distinguishing this from conventional NAFLD. Paper-13041665.
Citrullinemia is caused by either deficiency of argininosuccinate synthetase (ASS, citrullinemia type 1) or a defect of the SLC25A13 gene encoding a mitochondrial aspartate-glutamate transporter ( citrullinemia type II). Paper-10200661.
The increase in mitochondrial ATP levels caused by cell stimulation with Ca(2+)-mobilizing agonists was markedly larger in cells expressing aralar and citrin (but not truncated mutants lacking the Ca(2+)-binding site) than in control cells. Paper-10006375.
We have identified a new class of substrates, citrin and aralar1, which are Ca2+-binding aspartate/glutamate carriers (AGCs) of the mitochondrial inner membrane, using cross-linking and immunoprecipitation assays in isolated mitochondria. Paper-10535086.
Citrin, encoded by SLC25A13, is a liver-type mitochondrial aspartate-glutamate carrier (AGC), of which deficiency, in autosomal recessive trait, causes neonatal intrahepatic cholestasis (NICCD) and adult-onset type II citrullinemia (CTLN2). Paper-12102732.
We have previously reported that although citrin-knockout ( Ctrn(-/-)) mice fail to display symptoms of CTLN2, liver perfusion revealed a deficit in ureogenesis from ammonia accompanied by an increase in the perfusate lactate-to-pyruvate (L/P) ratio. Paper-11807450.
CTLN2 is associated with a high incidence of hepatocellular carcinoma ( HCC) in Japanese. We report a 48-year-old Taiwanese man with citrullinemia, who was in good health until the age of 34 when he had repeated episodes of consciousness disturbance. Paper-12245900.
BACKGROUND: Type II citrullinemia (CTLN2) characterized by a liver-specific argininosuccinate synthetase deficiency is an adult onset genetical disorder caused by the mutation of SLC25A13 gene, which results in fulminant hyperammonemia often with poor prognosis. Paper-8730862.
Adult-onset type II citrullinemia (CTLN2), characterized by a liver-specific deficiency of urea cycle enzyme, argininosuccinate synthetase, is caused by mutations in SLC25A13 that encodes a calcium binding mitochondrial solute carrier protein, citrin. Paper-9618273.
DESIGN: The effects of 3 different HCA-containing preparations (Regulator, Citrin K, Super CitriMax HCA-600-SXS, all used at an effective HCA dose of 150 and 300 mg/kg, administered intragastrically) on food intake and body weight were studied in adult male Wistar rats. Paper-11284809.
We examined the exons and intron/exon boundaries of the five key urea cycle enzymes, NAGS, and two solute carrier transporter genes ( SLC25A13 and SLC25A15) for sequence alterations using single-stranded conformational polymorphism ( SSCP) analysis and high-resolution melt profiling. Paper-13529627.
Citrin deficiency-associated fatty livers showed a considerably lower prevalence of accompanying obesity and metabolic syndrome, higher prevalence of history of pancreatitis, and higher serum levels of pancreatic secretory trypsin inhibitor ( PSTI) than fatty livers without the mutations. Paper-13041665.
Since the function of citrin, together with that of an isoform, aralar, was found to be as a mitochondrial aspartate glutamate carrier, the various symptoms of NICCD and CTLN2 may be understood as caused by defective aspartate export from the mitochondria to the cytosol and defects in the malate aspartate shuttle. Paper-9597441.
These synonyms are used for gene SLC25A13 (solute carrier family 25, member 13 (citrin)): Solute carrier family 25 member 13, Mitochondrial aspartate glutamate carrier 2, CTLN2, CITRIN, Citrin, Calcium-binding mitochondrial carrier protein Aralar2, ARALAR2.
These accession numbers are used for gene SLC25A13: Q9UNI7 (UNIPROT__AC), O14575 (UNIPROT__AC), AAF28473 (NCBI_GENBANK__AC), AAB67049 (NCBI_GENBANK__AC).
SLC25A13 is a homologue of SLC25A13 (solute carrier family 25, member 13 (citrin)) from Bos taurus.
SLC25A13 is a homologue of SLC25A13 (solute carrier family 25, member 13 (citrin)) from Pan troglodytes.
SLC25A13 is a homologue of SLC25A13 (solute carrier family 25, member 13 (citrin)) from Gallus gallus.
SLC25A13 is a homologue of SLC25A13 (solute carrier family 25, member 13 (citrin)) from Canis lupus familiaris.
SLC25A13 is a homologue of Slc25a13 (solute carrier family 25 (mitochondrial carrier, adenine nucleotide...) from Mus musculus.
SLC25A13 is a homologue of Slc25a13 (solute carrier family 25, member 13 (citrin)) from Rattus norvegicus.
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