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Search results 1 to 100 out of 372 for Otc

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0.043s
Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: chimpanzee
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: chicken
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: frog, western clawed
Gene
Type: gene
Organism: pig, domestic
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: dog, domestic
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Publication
First Author: Wang L
Year: 2017
Journal: Mol Genet Metab
Title: AAV gene therapy corrects OTC deficiency and prevents liver fibrosis in aged OTC-knock out heterozygous mice.
Volume: 120
Issue: 4
Pages: 299-305
Publication
First Author: Rivera-Barahona A
Year: 2015
Journal: PLoS One
Title: Functional characterization of the spf/ash splicing variation in OTC deficiency of mice and man.
Volume: 10
Issue: 4
Pages: e0122966
Publication
First Author: Luiking YC
Year: 2008
Journal: Am J Physiol Endocrinol Metab
Title: Reduced citrulline availability by OTC deficiency in mice is related to reduced nitric oxide production.
Volume: 295
Issue: 6
Pages: E1315-22
Publication  
First Author: Balestra D
Year: 2020
Journal: Int J Mol Sci
Title: An Exon-Specific Small Nuclear U1 RNA (ExSpeU1) Improves Hepatic OTC Expression in a Splicing-Defective spf/ash Mouse Model of Ornithine Transcarbamylase Deficiency.
Volume: 21
Issue: 22
Publication
First Author: Saheki T
Year: 1995
Journal: Biochim Biophys Acta
Title: Importance of ornithine transcarbamylase (OTC) deficiency in small intestine for urinary orotic acid excretion: analysis of OTC-deficient spf-ash mice with OTC transgene.
Volume: 1270
Issue: 1
Pages: 87-93
Publication
First Author: Zimmer KP
Year: 1999
Journal: Mol Med
Title: 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.
Volume: 5
Issue: 4
Pages: 244-53
Publication
First Author: Tarasenko TN
Year: 2015
Journal: PLoS One
Title: A new mouse model of mild ornithine transcarbamylase deficiency (spf-j) displays cerebral amino acid perturbations at baseline and upon systemic immune activation.
Volume: 10
Issue: 2
Pages: e0116594
Publication
First Author: Batshaw ML
Year: 1999
Journal: Pediatr Res
Title: Correction of ureagenesis after gene transfer in an animal model and after liver transplantation in humans with ornithine transcarbamylase deficiency.
Volume: 46
Issue: 5
Pages: 588-93
Publication
First Author: Wang L
Year: 2020
Journal: Sci Adv
Title: A mutation-independent CRISPR-Cas9-mediated gene targeting approach to treat a murine model of ornithine transcarbamylase deficiency.
Volume: 6
Issue: 7
Pages: eaax5701
Publication
First Author: Rosenberg LE
Year: 1983
Journal: Science
Title: Biogenesis of ornithine transcarbamylase in spfash mutant mice: two cytoplasmic precursors, one mitochondrial enzyme.
Volume: 222
Issue: 4622
Pages: 426-8
Publication
First Author: Cavard C
Year: 1988
Journal: Nucleic Acids Res
Title: Correction of mouse ornithine transcarbamylase deficiency by gene transfer into the germ line.
Volume: 16
Issue: 5
Pages: 2099-110
Publication
First Author: Rao VL
Year: 1993
Journal: Pediatr Res
Title: Increased densities of binding sites for the peripheral-type benzodiazepine receptor ligand [3H]PK 11195 in congenital ornithine transcarbamylase-deficient sparse fur mouse.
Volume: 34
Issue: 6
Pages: 777-80
Publication
First Author: Davies KE
Year: 1985
Journal: Nucleic Acids Res
Title: Gene for OTC: characterisation and linkage to Duchenne muscular dystrophy.
Volume: 13
Issue: 1
Pages: 155-65
Publication
First Author: Grompe M
Year: 1992
Journal: Hum Gene Ther
Title: Retroviral-mediated gene transfer of human ornithine transcarbamylase into primary hepatocytes of spf and spf-ash mice.
Volume: 3
Issue: 1
Pages: 35-44
Publication  
First Author: Shimada T
Year: 1994
Journal: J Dermatol Sci
Title: Normalization of hair growth in sparse fur-abnormal skin and hair (SPF-ASH) mice by introduction of the rat ornithine transcarbamylase (OTC) gene.
Volume: 7 Suppl
Pages: S27-32
Publication
First Author: Marini JC
Year: 2006
Journal: J Nutr
Title: Reduced ornithine transcarbamylase activity does not impair ureagenesis in Otc(spf-ash) mice.
Volume: 136
Issue: 4
Pages: 1017-20
Publication
First Author: Bell P
Year: 2006
Journal: Mol Ther
Title: Analysis of tumors arising in male B6C3F1 mice with and without AAV vector delivery to liver.
Volume: 14
Issue: 1
Pages: 34-44
Publication
First Author: Qureshi IA
Year: 1985
Journal: J Pediatr Gastroenterol Nutr
Title: Expression of ornithine transcarbamylase deficiency in the small intestine and colon of sparse-fur mutant mice.
Volume: 4
Issue: 1
Pages: 118-24
Publication
First Author: Rao KV
Year: 1997
Journal: Neurosci Lett
Title: Progressive decrease of cerebral cytochrome C oxidase activity in sparse-fur mice: role of acetyl-L-carnitine in restoring the ammonia-induced cerebral energy depletion.
Volume: 224
Issue: 2
Pages: 83-6
Publication
First Author: Monastiri K
Year: 1993
Journal: Prenat Diagn
Title: Prenatal diagnosis of ornithine transcarbamylase deficiency: results in Spfash mice.
Volume: 13
Issue: 6
Pages: 441-7
Publication
First Author: Ratnakumari L
Year: 1994
Journal: Metab Brain Dis
Title: Regional amino acid neurotransmitter changes in brains of spf/Y mice with congenital ornithine transcarbamylase deficiency.
Volume: 9
Issue: 1
Pages: 43-51
Publication
First Author: Ratnakumari L
Year: 1996
Journal: Pediatr Res
Title: Central muscarinic cholinergic M1 and M2 receptor changes in congenital ornithine transcarbamylase deficiency.
Volume: 40
Issue: 1
Pages: 25-8
Publication
First Author: Kiwaki K
Year: 1996
Journal: Hum Gene Ther
Title: Correction of ornithine transcarbamylase deficiency in adult spf(ash) mice and in OTC-deficient human hepatocytes with recombinant adenoviruses bearing the CAG promoter.
Volume: 7
Issue: 7
Pages: 821-30
Publication  
First Author: Mullins LJ
Year: 1986
Journal: Mouse News Lett
Title: Polymorphism of the ornithine transcarboxylase locus and control of gene expression.
Volume: 74
Pages: 114
Publication
First Author: Malo C
Year: 1994
Journal: Comp Biochem Physiol A Physiol
Title: Free amino acid levels in serum and small intestine during the post-natal development of normal and sparse-fur mutant mice.
Volume: 109
Issue: 4
Pages: 1049-57
Publication
First Author: Ratnakumari L
Year: 1994
Journal: Neurosci Lett
Title: Evidence for cholinergic neuronal loss in brain in congenital ornithine transcarbamylase deficiency.
Volume: 178
Issue: 1
Pages: 63-5
Publication
First Author: García-Pérez MA
Year: 1995
Journal: Hum Genet
Title: Demonstration of the spf-ash mutation in Spanish patients with ornithine transcarbamylase deficiency of moderate severity.
Volume: 95
Issue: 2
Pages: 183-6
Publication  
First Author: Evans EP
Year: 1987
Journal: Mouse News Lett
Title: A repositioning of the X Chromosome breakpoint of T(X;11)38H.
Volume: 77
Pages: 147
Publication
First Author: Soria LR
Year: 2021
Journal: EMBO Mol Med
Title: Beclin-1-mediated activation of autophagy improves proximal and distal urea cycle disorders.
Volume: 13
Issue: 2
Pages: e13158
Publication
First Author: McGuire PJ
Year: 2014
Journal: Dis Model Mech
Title: Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency.
Volume: 7
Issue: 2
Pages: 205-13
Publication
First Author: Nelson J
Year: 1993
Journal: Chem Biol Interact
Title: The effects of various inhibitors on the regulation of orotic acid excretion in sparse-fur mutant mice (spf/Y) deficient in ornithine transcarbamylase.
Volume: 89
Issue: 1
Pages: 35-47
Publication
First Author: Santamaria R
Year: 2022
Journal: Sci Rep
Title: Derivation of healthy hepatocyte-like cells from a female patient with ornithine transcarbamylase deficiency through X-inactivation selection.
Volume: 12
Issue: 1
Pages: 2308
Publication
First Author: Laval SH
Year: 1993
Journal: Mamm Genome
Title: Partial inversion of gene order within a homologous segment on the X chromosome.
Volume: 4
Issue: 2
Pages: 119-23
Publication
First Author: Yan D
Year: 1998
Journal: J Biol Chem
Title: Biochemical characterization and subcellular localization of the mouse retinitis pigmentosa GTPase regulator (mRpgr).
Volume: 273
Issue: 31
Pages: 19656-63
Publication
First Author: Inoue Y
Year: 2002
Journal: J Biol Chem
Title: Defective ureagenesis in mice carrying a liver-specific disruption of hepatocyte nuclear factor 4alpha (HNF4alpha ). HNF4alpha regulates ornithine transcarbamylase in vivo.
Volume: 277
Issue: 28
Pages: 25257-65
Publication
First Author: Chapman VM
Year: 1991
Journal: Genomics
Title: Linkage of the erythroid transcription factor gene (Gf-1) to the proximal region of the X chromosome of mice.
Volume: 9
Issue: 2
Pages: 309-13
Publication
First Author: Grant SG
Year: 1991
Journal: Oncogene
Title: Detailed genetic mapping of the A-raf proto-oncogene on the mouse X chromosome.
Volume: 6
Issue: 3
Pages: 397-402
Publication
First Author: Shiojiri N
Year: 2001
Journal: Lab Invest
Title: Cell lineage analysis during liver development using the spf(ash)-heterozygous mouse.
Volume: 81
Issue: 1
Pages: 17-25
Publication
First Author: Shiojiri N
Year: 2000
Journal: Am J Pathol
Title: Quantitative analysis of cell allocation during liver development, using the spf(ash)-heterozygous female mouse.
Volume: 156
Issue: 1
Pages: 65-75
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: Veres G
Year: 1987
Journal: Science
Title: The molecular basis of the sparse fur mouse mutation.
Volume: 237
Issue: 4813
Pages: 415-7
Publication
First Author: Scherer SE
Year: 1988
Journal: Nucleic Acids Res
Title: The genetic structure of mouse ornithine transcarbamylase.
Volume: 16
Issue: 4
Pages: 1593-601
Publication
First Author: Lichter-Konecki U
Year: 2008
Journal: Glia
Title: Gene expression profiling of astrocytes from hyperammonemic mice reveals altered pathways for water and potassium homeostasis in vivo.
Volume: 56
Issue: 4
Pages: 365-77
Publication  
First Author: Hunsicker P
Year: 1974
Journal: Mouse News Lett
Title: Ie = eye-ear reduction X linked.
Volume: 50
Pages: 51-2
Publication
First Author: Rangroo Thrane V
Year: 2013
Journal: Nat Med
Title: Ammonia triggers neuronal disinhibition and seizures by impairing astrocyte potassium buffering.
Volume: 19
Issue: 12
Pages: 1643-8
Publication
First Author: Doolittle DP
Year: 1974
Journal: J Hered
Title: A new allele of the sparse fur gene in the mouse.
Volume: 65
Issue: 3
Pages: 194-5
Publication
First Author: Logan GJ
Year: 2017
Journal: Nat Genet
Title: Identification of liver-specific enhancer-promoter activity in the 3' untranslated region of the wild-type AAV2 genome.
Volume: 49
Issue: 8
Pages: 1267-1273
Publication
First Author: Marini JC
Year: 2006
Journal: J Nutr
Title: Ornithine restores ureagenesis capacity and mitigates hyperammonemia in Otc(spf-ash) mice.
Volume: 136
Issue: 7
Pages: 1834-8
Publication
First Author: Marini JC
Year: 2006
Journal: Mol Genet Metab
Title: Hyperammonemia increases sensitivity to LPS.
Volume: 88
Issue: 2
Pages: 131-7
Publication
First Author: Shiojiri N
Year: 2003
Journal: Histochem Cell Biol
Title: Mosaic analysis of small intestinal development using the spf(ash)-heterozygous female mouse.
Volume: 119
Issue: 3
Pages: 199-210
Publication
First Author: Marini JC
Year: 2007
Journal: Am J Physiol Endocrinol Metab
Title: Interaction between murine spf-ash mutation and genetic background yields different metabolic phenotypes.
Volume: 293
Issue: 6
Pages: E1764-71
Publication  
First Author: Kurtz CB
Year: 2019
Journal: Sci Transl Med
Title: An engineered E. coli Nissle improves hyperammonemia and survival in mice and shows dose-dependent exposure in healthy humans.
Volume: 11
Issue: 475
Publication
First Author: Li MX
Year: 1999
Journal: Biochim Biophys Acta
Title: 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.
Volume: 1455
Issue: 1
Pages: 1-11
Publication  
First Author: Cavino K
Year: 2021
Journal: Endocrinology
Title: Glucagon Receptor Inhibition Reduces Hyperammonemia and Lethality in Male Mice with Urea Cycle Disorder.
Volume: 162
Issue: 1
Publication
First Author: Hodges PE
Year: 1989
Journal: Proc Natl Acad Sci U S A
Title: The spfash mouse: a missense mutation in the ornithine transcarbamylase gene also causes aberrant mRNA splicing.
Volume: 86
Issue: 11
Pages: 4142-6
Publication
First Author: Cohen NS
Year: 1989
Journal: Biochem J
Title: Altered enzyme activities and citrulline synthesis in liver mitochondria from ornithine carbamoyltransferase-deficient sparse-furash mice.
Volume: 257
Issue: 1
Pages: 251-7
Publication
First Author: Shiojiri N
Year: 1997
Journal: Am J Pathol
Title: Mosaic pattern of ornithine transcarbamylase expression in spfash mouse liver.
Volume: 151
Issue: 2
Pages: 413-21
Publication  
First Author: Cupp MB
Year: 1958
Journal: Mouse News Lett
Title: Sparse-fur, Sf
Volume: 19
Pages: 37
Publication
First Author: Hopkins KJ
Year: 1998
Journal: Brain Res
Title: Dendritic alterations in cortical pyramidal cells in the sparse fur mouse.
Volume: 797
Issue: 1
Pages: 167-72
Publication
First Author: D'Hooge R
Year: 2000
Journal: Brain Res
Title: Impaired cognitive performance in ornithine transcarbamylase-deficient mice on arginine-free diet.
Volume: 876
Issue: 1-2
Pages: 1-9
Publication
First Author: Ratnakumari L
Year: 1993
Journal: Biochem Pharmacol
Title: Effect of sodium benzoate on cerebral and hepatic energy metabolites in spf mice with congenital hyperammonemia.
Volume: 45
Issue: 1
Pages: 137-46
Publication
First Author: DeMars R
Year: 1976
Journal: Proc Natl Acad Sci U S A
Title: Abnormal ornithine carbamoyltransferase in mice having the sparse-fur mutation.
Volume: 73
Issue: 5
Pages: 1693-7
Publication
First Author: Batshaw ML
Year: 1995
Journal: Gene Ther
Title: The sparse fur mouse as a model for gene therapy in ornithine carbamoyltransferase deficiency.
Volume: 2
Issue: 10
Pages: 743-9
Publication
First Author: Ratnakumari L
Year: 1995
Journal: J Pharmacol Exp Ther
Title: Developmental deficiency of the cholinergic system in congenitally hyperammonemic spf mice: effect of acetyl-L-carnitine.
Volume: 274
Issue: 1
Pages: 437-43
Publication
First Author: Robinson MB
Year: 1992
Journal: J Neurochem
Title: Brain serotonin2 and serotonin1A receptors are altered in the congenitally hyperammonemic sparse fur mouse.
Volume: 58
Issue: 3
Pages: 1016-22
Publication
First Author: Vasudevan S
Year: 1992
Journal: Biochem Med Metab Biol
Title: Abnormal hepatic nucleotide pools in sparse fur (spf) mutant mice deficient in ornithine transcarbamylase.
Volume: 47
Issue: 3
Pages: 274-8
Publication
First Author: Briand P
Year: 1981
Journal: FEBS Lett
Title: Increase of ornithine transcarbamylase protein in sparse-fur mice with ornithine transcarbamylase deficiency.
Volume: 130
Issue: 1
Pages: 65-8
Publication
First Author: Ratnakumari L
Year: 1992
Journal: Biochem Biophys Res Commun
Title: Effects of congenital hyperammonemia on the cerebral and hepatic levels of the intermediates of energy metabolism in spf mice.
Volume: 184
Issue: 2
Pages: 746-51
Publication
First Author: Hopkins KJ
Year: 1998
Journal: Brain Res
Title: Characterization of N-methyl-d-aspartate receptors in the hyperammonemic sparse fur mouse.
Volume: 797
Issue: 2
Pages: 209-17
Publication
First Author: Briand P
Year: 1982
Journal: Biochim Biophys Acta
Title: Ornithine transcarbamylase deficiencies in human males. Kinetic and immunochemical classification.
Volume: 704
Issue: 1
Pages: 100-6
Publication  
First Author: Lyon MF
Year: 1986
Journal: J Embryol Exp Morphol
Title: Lack of inactivation of a mouse X-linked gene physically separated from the inactivation centre.
Volume: 97
Pages: 75-85