151 related articles for article (PubMed ID: 36220785)
1. Excretion of excess nitrogen and increased survival by loss of SLC6A19 in a mouse model of ornithine transcarbamylase deficiency.
Belanger AJ; Gefteas E; Przybylska M; Geller S; Anarat-Cappillino G; Kloss A; Yew NS
J Inherit Metab Dis; 2023 Jan; 46(1):55-65. PubMed ID: 36220785
[TBL] [Abstract][Full Text] [Related]
2. Comprehensive characterization of ureagenesis in the spf
Allegri G; Deplazes S; Rimann N; Causton B; Scherer T; Leff JW; Diez-Fernandez C; Klimovskaia A; Fingerhut R; Krijt J; Kožich V; Nuoffer JM; Grisch-Chan HM; Thöny B; Häberle J
J Inherit Metab Dis; 2019 Nov; 42(6):1064-1076. PubMed ID: 30714172
[TBL] [Abstract][Full Text] [Related]
3. Corticosteroid suppresses urea-cycle-related gene expressions in ornithine transcarbamylase deficiency.
Imoto K; Tanaka M; Goya T; Aoyagi T; Takahashi M; Kurokawa M; Tashiro S; Kato M; Kohjima M; Ogawa Y
BMC Gastroenterol; 2022 Mar; 22(1):144. PubMed ID: 35346058
[TBL] [Abstract][Full Text] [Related]
4. Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency.
McGuire PJ; Tarasenko TN; Wang T; Levy E; Zerfas PM; Moran T; Lee HS; Bequette BJ; Diaz GA
Dis Model Mech; 2014 Feb; 7(2):205-13. PubMed ID: 24271778
[TBL] [Abstract][Full Text] [Related]
5. Ornithine restores ureagenesis capacity and mitigates hyperammonemia in Otc(spf-ash) mice.
Marini JC; Lee B; Garlick PJ
J Nutr; 2006 Jul; 136(7):1834-8. PubMed ID: 16772445
[TBL] [Abstract][Full Text] [Related]
6. Interaction between murine spf-ash mutation and genetic background yields different metabolic phenotypes.
Marini JC; Erez A; Castillo L; Lee B
Am J Physiol Endocrinol Metab; 2007 Dec; 293(6):E1764-71. PubMed ID: 17925451
[TBL] [Abstract][Full Text] [Related]
7. Glutaminase 2 knockdown reduces hyperammonemia and associated lethality of urea cycle disorder mouse model.
Mao X; Chen H; Lin AZ; Kim S; Burczynski ME; Na E; Halasz G; Sleeman MW; Murphy AJ; Okamoto H; Cheng X
J Inherit Metab Dis; 2022 May; 45(3):470-480. PubMed ID: 34988999
[TBL] [Abstract][Full Text] [Related]
8. Reduced ornithine transcarbamylase activity does not impair ureagenesis in Otc(spf-ash) mice.
Marini JC; Lee B; Garlick PJ
J Nutr; 2006 Apr; 136(4):1017-20. PubMed ID: 16549467
[TBL] [Abstract][Full Text] [Related]
9. 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.
Li MX; Nakajima T; Fukushige T; Kobayashi K; Seiler N; Saheki T
Biochim Biophys Acta; 1999 Sep; 1455(1):1-11. PubMed ID: 10524224
[TBL] [Abstract][Full Text] [Related]
10. Inhibiting neutral amino acid transport for the treatment of phenylketonuria.
Belanger AM; Przybylska M; Gefteas E; Furgerson M; Geller S; Kloss A; Cheng SH; Zhu Y; Yew NS
JCI Insight; 2018 Jul; 3(14):. PubMed ID: 30046012
[TBL] [Abstract][Full Text] [Related]
11. Phenylbutyrate improves nitrogen disposal via an alternative pathway without eliciting an increase in protein breakdown and catabolism in control and ornithine transcarbamylase-deficient patients.
Marini JC; Lanpher BC; Scaglia F; O'Brien WE; Sun Q; Garlick PJ; Jahoor F; Lee B
Am J Clin Nutr; 2011 Jun; 93(6):1248-54. PubMed ID: 21490144
[TBL] [Abstract][Full Text] [Related]
12. Efficient mitochondrial import of newly synthesized ornithine transcarbamylase (OTC) and correction of secondary metabolic alterations in spf(ash) mice following gene therapy of OTC deficiency.
Zimmer KP; Bendiks M; Mori M; Kominami E; Robinson MB; Ye X; Wilson JM
Mol Med; 1999 Apr; 5(4):244-53. PubMed ID: 10448647
[TBL] [Abstract][Full Text] [Related]
13. A new mouse model of mild ornithine transcarbamylase deficiency (spf-j) displays cerebral amino acid perturbations at baseline and upon systemic immune activation.
Tarasenko TN; Rosas OR; Singh LN; Kristaponis K; Vernon H; McGuire PJ
PLoS One; 2015; 10(2):e0116594. PubMed ID: 25647322
[TBL] [Abstract][Full Text] [Related]
14. The sparse fur mouse as a model for gene therapy in ornithine carbamoyltransferase deficiency.
Batshaw ML; Yudkoff M; McLaughlin BA; Gorry E; Anegawa NJ; Smith IA; Hyman SL; Robinson MB
Gene Ther; 1995 Dec; 2(10):743-9. PubMed ID: 8750014
[TBL] [Abstract][Full Text] [Related]
15. Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients.
Scaglia F; Carter S; O'Brien WE; Lee B
Mol Genet Metab; 2004 Apr; 81 Suppl 1():S79-85. PubMed ID: 15050979
[TBL] [Abstract][Full Text] [Related]
16. The dogfish shark (Squalus acanthias) increases both hepatic and extrahepatic ornithine urea cycle enzyme activities for nitrogen conservation after feeding.
Kajimura M; Walsh PJ; Mommsen TP; Wood CM
Physiol Biochem Zool; 2006; 79(3):602-13. PubMed ID: 16691526
[TBL] [Abstract][Full Text] [Related]
17. Blood levels of ammonia and nitrogen scavenging amino acids in patients with inherited hyperammonemia.
Tuchman M; Yudkoff M
Mol Genet Metab; 1999 Jan; 66(1):10-5. PubMed ID: 9973542
[TBL] [Abstract][Full Text] [Related]
18. Importance of ornithine transcarbamylase (OTC) deficiency in small intestine for urinary orotic acid excretion: analysis of OTC-deficient spf-ash mice with OTC transgene.
Saheki T; Mori K; Kobayashi K; Horiuchi M; Shige T; Obara T; Suzuki S; Mori M; Yamamura K
Biochim Biophys Acta; 1995 Jan; 1270(1):87-93. PubMed ID: 7827141
[TBL] [Abstract][Full Text] [Related]
19. Molecular basis of ornithine transcarbamylase deficiency in spf and spf-ash mutant mice.
Ohtake A; Takayanagi M; Yamamoto S; Kakinuma H; Nakajima H; Tatibana M; Mori M
J Inherit Metab Dis; 1986; 9(3):289-91. PubMed ID: 3099076
[No Abstract] [Full Text] [Related]
20. Prenatal treatment of ornithine transcarbamylase deficiency.
Wilnai Y; Blumenfeld YJ; Cusmano K; Hintz SR; Alcorn D; Benitz WE; Berquist WE; Bernstein JA; Castillo RO; Concepcion W; Cowan TM; Cox KL; Lyell DJ; Esquivel CO; Homeyer M; Hudgins L; Hurwitz M; Palma JP; Schelley S; Akula VP; Summar ML; Enns GM
Mol Genet Metab; 2018 Mar; 123(3):297-300. PubMed ID: 29396029
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
