|1.||Tuchman, Mendel: 8 articles (05/2015 - 02/2002)|
|2.||Häberle, Johannes: 5 articles (11/2015 - 06/2003)|
|3.||Yoo, Han-Wook: 4 articles (09/2015 - 11/2006)|
|4.||Morizono, Hiroki: 4 articles (05/2015 - 02/2002)|
|5.||Murayama, Hiroshi: 4 articles (01/2014 - 05/2007)|
|6.||Lee, Brendan: 4 articles (09/2013 - 09/2004)|
|7.||Wilson, James M: 4 articles (09/2013 - 09/2003)|
|8.||Batshaw, Mark L: 4 articles (09/2013 - 09/2003)|
|9.||Lichter-Konecki, Uta: 4 articles (03/2009 - 03/2008)|
|10.||Choi, Jin-Ho: 3 articles (09/2015 - 11/2006)|
|1.||Ornithine Carbamoyltransferase Deficiency Disease
02/16/1996 - "A murine model of ornithine transcarbamylase (OTC) deficiency was used in this study to evaluate the efficacy of recombinant adenoviruses for correcting the metabolic defect in liver. "
06/10/2001 - "Mouse models of ornithine transcarbamylase (OTC) deficiency are being used to test the efficacy of viral vectors as possible vehicles for gene therapy. "
09/01/2013 - "A 66-year-old woman heterozygous for a mutation in the ornithine transcarbamylase gene (Otc) participated in a phase I gene therapy trial for OTC deficiency. "
05/01/2011 - "This study aimed at understanding clinical features, biochemistry and gene mutation in one Chinese pedigree which had a neonatal-onset ornithine transcarbamylase deficiency (OTCD) boy, and exploring the significance of ornithine transcarbamylase analysis in prenatal diagnosis. "
06/01/2009 - "For ornithine transcarbamylase (OTC) deficiency, in vitro studies have suggested the presence of dominant-negative effects, however, supporting in vivo studies have not been conducted. "
11/01/2006 - "Immune IgA was greatly increased in the serum of pIgR-deficient mice, conferred passive protection against Giardia, and recognized several conserved giardial Ags, including ornithine carbamoyltransferase, arginine deiminase, alpha-enolase, and alpha- and beta-giardins, that are also detected in human giardiasis. "
|3.||Reye Syndrome (Reye's Syndrome)
09/25/1977 - "This study indicated that the reduced level of ornithine transcarbamylase activity commonly observed in Reye's syndrome is not necessarily due to structural or functional alterations of the enzyme."
10/01/1991 - "In two patients with fatal Reye's Syndrome, total ornithine carbamoyltransferase (OCTase) activity in the liver was 50 and 75% of that found in three control livers. "
10/01/1991 - "Displacement of hepatic ornithine carbamoyltransferase from mitochondria to cytosol in Reye's syndrome."
11/26/1982 - "Pathological aspects of ornithine transcarbamylase including the enzyme deficiency and reduced activities of the enzyme in Reye's syndrome are also described. "
09/25/1977 - "Ornithine transcarbamylase from normal liver has an isoelectric point of 7.95, and the value for the enzyme from the Reye's syndrome liver was 8.05. "
|4.||Inborn Urea Cycle Disorders
07/24/1987 - "The ornithine transcarbamylase-deficient sparse fur mouse is an excellent model to study the most common human urea cycle disorder. "
08/01/2003 - "Despite the apparent functional ablation of ORNT1 in 10 French-Canadian probands with the ORNT1-F188 Delta allele, these patients are mildly affected when compared to patients with other urea cycle disorders such as deficiency of ornithine transcarbamylase. "
01/01/2002 - "Ornithine carbamoyltransferase (OCT) deficiency is the commonest of the inherited urea cycle disorders. "
01/01/1998 - "Ornithine transcarbamylase (OTCase) deficiency, the most common inherited urea cycle disorder, is transmitted as an X-linked trait. "
09/15/2013 - "Ornithine transcarbamylase (OTC) deficiency is the most common urea cycle disorder and demonstrates X-linked inheritance. "
10/21/1992 - "On chromosome Xp, tumor-specific allele loss was observed in 9 out of 15 informative cases (60%) at the ornithine transcarbamylase (OTC) gene locus. "
05/01/2013 - "Here we report that in rAAV-treated ornithine transcarbamylase (Otc)-deficient mice, four examples of integration sites in Dlk1-Dio3 could be detected in specimens from liver nodule/tumors, confirming previous studies of rAAV integration in the Dlk1-Dio3 locus in the setting of another murine model of metabolic disease. "
03/01/1982 - "It did not cleave several of the protein and peptide substrates tested including the precursor of mitochondrial carbamoyl-phosphate synthetase I. Apparently the same protease activity is widely distributed among mitochondria of rat kidney, spleen, heart and ascites tumor cells, all of which lack ornithine carbamoyltransferase. "
10/01/1982 - "Lowered level of translatable messenger RNAs for carbamyl phosphate synthetase-I and ornithine transcarbamylase in the liver of tumor-bearing mice."
10/01/1982 - "The activities of two urea cycle enzymes, carbamyl phosphate synthetase-I and ornithine transcarbamylase, in mouse liver gradually decreased after transplantation of Ehrlich tumor cells into the subepidermal tissue, and reached values of 60 and 65% of those of control mice, respectively, on the 21st day. "
|1.||Complementary DNA (cDNA)
|4.||Phosphopyruvate Hydratase (Enolase)
|5.||Immunoglobulin A (IgA)
|7.||Carbamyl Phosphate (Carbamoyl Phosphate)
|9.||DNA (Deoxyribonucleic Acid)
|1.||Transplantation (Transplant Recipients)
|4.||Carbohydrate-Restricted Diet (Low Carbohydrate Diet)