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Search results 1 to 100 out of 615 for Pah

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Type Details Score
Gene
Type: gene
Organism: Homo sapiens
Gene
Type: gene
Organism: Rattus norvegicus
Gene
Type: gene
Organism: Danio rerio
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Domain
Type: Repeat
Description: This entry represents the paired amphipathic helix (PAH) repeat. Sin3 proteins have at least three PAH domains (PAH1, PAH2, and PAH3) [, ]. They are components of a co-repressor complex that silences transcription, playing important roles in the transition between proliferation and differentiation. Sin3 proteins are recruited to the DNA by various DNA-binding transcription factors such as the Mad family of repressors, Mnt/Rox, PLZF, MeCP2, p53, REST/NRSF, MNFbeta, Sp1, TGIF and Ume6 []. Sin3 acts as a scaffold protein that in turn recruits histone-binding proteins RbAp46/RbAp48 and histone deacetylases HDAC1/HDAC2, which deacetylate the core histones resulting in a repressed state of the chromatin []. The PAH domains are protein-protein interaction domains through which Sin3 fulfils its role as a scaffold. The PAH2 domain of Sin3 can interact with a wide range of unrelated and structurally diverse transcription factors that bind using different interaction motifs. For example, the Sin3 PAH2 domain can interact with the unrelated Mad and HBP1 factors using alternative interaction motifs that involve binding in opposite helical orientations [].
Publication
First Author: Lagler FB
Year: 2010
Journal: Biochem Pharmacol
Title: New insights into tetrahydrobiopterin pharmacodynamics from Pah enu1/2, a mouse model for compound heterozygous tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.
Volume: 80
Issue: 10
Pages: 1563-71
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1689419
Stage: TS19
Assay Id: MGI:6191012
Age: embryonic day 11.5
Specimen Label: Table S2 - E11.5 - Pah
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1689421
Stage: TS21
Assay Id: MGI:6191012
Age: embryonic day 13.5
Specimen Label: Table S2 - E13.5 - Pah
Detected: false
Specimen Num: 2
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Male
Emaps: EMAPS:1689424
Stage: TS24
Assay Id: MGI:6191012
Age: embryonic day 15.5
Specimen Label: Table S2 - E15.5 - Pah
Detected: false
Specimen Num: 3
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Male
Emaps: EMAPS:1689426
Stage: TS26
Assay Id: MGI:6191012
Age: embryonic day 18.5
Specimen Label: Table S2 - E18.5 - Pah
Detected: false
Specimen Num: 4
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Male
Emaps: EMAPS:1689428
Stage: TS28
Assay Id: MGI:6191012
Age: postnatal day 4
Specimen Label: Table S2 - P4 - Pah
Detected: false
Specimen Num: 5
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Male
Emaps: EMAPS:1689428
Stage: TS28
Assay Id: MGI:6191012
Age: postnatal day 14
Specimen Label: Table S2 - P14 - Pah
Detected: false
Specimen Num: 6
GXD Expression      
Probe: MGI:6180008
Assay Type: RNA in situ
Annotation Date: 2018-07-25
Strength: Absent
Sex: Male
Emaps: EMAPS:1689428
Stage: TS28
Assay Id: MGI:6191012
Age: postnatal day 28
Specimen Label: Table S2 - P28 - Pah
Detected: false
Specimen Num: 7
Publication
First Author: Spronk CA
Year: 2000
Journal: Nat Struct Biol
Title: The Mad1-Sin3B interaction involves a novel helical fold.
Volume: 7
Issue: 12
Pages: 1100-4
Protein
Organism: Mus musculus
Length: 133  
Fragment?: true
Publication
First Author: Swanson KA
Year: 2004
Journal: Nat Struct Mol Biol
Title: HBP1 and Mad1 repressors bind the Sin3 corepressor PAH2 domain with opposite helical orientations.
Volume: 11
Issue: 8
Pages: 738-46
Protein
Organism: Mus musculus
Length: 293  
Fragment?: false
Protein
Organism: Mus musculus
Length: 190  
Fragment?: true
Protein
Organism: Mus musculus
Length: 207  
Fragment?: true
Publication
First Author: van Ingen H
Year: 2004
Journal: Biochemistry
Title: Extension of the binding motif of the Sin3 interacting domain of the Mad family proteins.
Volume: 43
Issue: 1
Pages: 46-54
Publication
First Author: Sahu SC
Year: 2008
Journal: J Mol Biol
Title: Conserved themes in target recognition by the PAH1 and PAH2 domains of the Sin3 transcriptional corepressor.
Volume: 375
Issue: 5
Pages: 1444-56
Protein
Organism: Mus musculus
Length: 174  
Fragment?: false
Protein
Organism: Mus musculus
Length: 167  
Fragment?: true
Publication
First Author: Kumar GS
Year: 2011
Journal: J Mol Biol
Title: Solution structure of the mSin3A PAH2-Pf1 SID1 complex: a Mad1/Mxd1-like interaction disrupted by MRG15 in the Rpd3S/Sin3S complex.
Volume: 408
Issue: 5
Pages: 987-1000
Protein
Organism: Mus musculus
Length: 1274  
Fragment?: false
Protein
Organism: Mus musculus
Length: 1098  
Fragment?: false
Protein
Organism: Mus musculus
Length: 1098  
Fragment?: false
Protein
Organism: Mus musculus
Length: 2260  
Fragment?: false
Protein
Organism: Mus musculus
Length: 2036  
Fragment?: true
Protein
Organism: Mus musculus
Length: 2242  
Fragment?: false
Protein
Organism: Mus musculus
Length: 2243  
Fragment?: false
Publication
First Author: Harding CO
Year: 1998
Journal: Gene Ther
Title: Metabolic engineering as therapy for inborn errors of metabolism--development of mice with phenylalanine hydroxylase expression in muscle.
Volume: 5
Issue: 5
Pages: 677-83
Publication
First Author: Gunasekera RS
Year: 2009
Journal: Mol Genet Metab
Title: In vivo regulation of phenylalanine hydroxylase in the genetic mutant hph-1 mouse model.
Volume: 98
Issue: 3
Pages: 264-72
Publication
First Author: Ledley FD
Year: 1990
Journal: Biochem J
Title: Mouse phenylalanine hydroxylase. Homology and divergence from human phenylalanine hydroxylase.
Volume: 267
Issue: 2
Pages: 399-405
Publication
First Author: Eichinger A
Year: 2018
Journal: Hum Mol Genet
Title: Secondary BH4 deficiency links protein homeostasis to regulation of phenylalanine metabolism.
Volume: 27
Issue: 10
Pages: 1732-1742
Publication
First Author: Liu TJ
Year: 1992
Journal: Somat Cell Mol Genet
Title: Reconstitution of enzymatic activity in hepatocytes of phenylalanine hydroxylase-deficient mice.
Volume: 18
Issue: 1
Pages: 89-96
Publication
First Author: Aubi O
Year: 2021
Journal: Nat Commun
Title: The Pah-R261Q mouse reveals oxidative stress associated with amyloid-like hepatic aggregation of mutant phenylalanine hydroxylase.
Volume: 12
Issue: 1
Pages: 2073
Publication
First Author: Sarkissian CN
Year: 2000
Journal: Mol Genet Metab
Title: A heteroallelic mutant mouse model: A new orthologue for human hyperphenylalaninemia.
Volume: 69
Issue: 3
Pages: 188-94
Publication  
First Author: Oh HJ
Year: 2005
Journal: Mol Genet Metab
Title: Reversal of gene expression profile in the phenylketonuria mouse model after adeno-associated virus vector-mediated gene therapy.
Volume: 86 Suppl 1
Pages: S124-32
Publication
First Author: Zagreda L
Year: 1999
Journal: J Neurosci
Title: Cognitive deficits in a genetic mouse model of the most common biochemical cause of human mental retardation.
Volume: 19
Issue: 14
Pages: 6175-82
Publication
First Author: Haefele MJ
Year: 2001
Journal: Mol Genet Metab
Title: Characterization of the mouse phenylalanine hydroxylase mutation Pah(enu3).
Volume: 72
Issue: 1
Pages: 27-30
Publication
First Author: Singh K
Year: 2021
Journal: Sci Rep
Title: CRISPR/Cas9 generated knockout mice lacking phenylalanine hydroxylase protein as a novel preclinical model for human phenylketonuria.
Volume: 11
Issue: 1
Pages: 7254
Publication
First Author: Faust DM
Year: 1990
Journal: Differentiation
Title: Activation of phenylalanine hydroxylase expression following genomic DNA transfection of hepatoma cells.
Volume: 44
Issue: 1
Pages: 74-9
Publication
First Author: Kure S
Year: 2004
Journal: Mol Genet Metab
Title: Wild-type phenylalanine hydroxylase activity is enhanced by tetrahydrobiopterin supplementation in vivo: an implication for therapeutic basis of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency.
Volume: 83
Issue: 1-2
Pages: 150-6
Publication  
First Author: Richards DY
Year: 2020
Journal: Mol Ther Methods Clin Dev
Title: AAV-Mediated CRISPR/Cas9 Gene Editing in Murine Phenylketonuria.
Volume: 17
Pages: 234-245
Publication
First Author: Bell SM
Year: 2017
Journal: PLoS One
Title: Formulation and PEGylation optimization of the therapeutic PEGylated phenylalanine ammonia lyase for the treatment of phenylketonuria.
Volume: 12
Issue: 3
Pages: e0173269
Publication
First Author: Gersting SW
Year: 2010
Journal: Hum Mol Genet
Title: Pahenu1 is a mouse model for tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency and promotes analysis of the pharmacological chaperone mechanism in vivo.
Volume: 19
Issue: 10
Pages: 2039-49
Publication
First Author: Bode VC
Year: 1988
Journal: Genetics
Title: hph-1: a mouse mutant with hereditary hyperphenylalaninemia induced by ethylnitrosourea mutagenesis.
Volume: 118
Issue: 2
Pages: 299-305
Publication
First Author: Ding Z
Year: 2006
Journal: Gene Ther
Title: Administration-route and gender-independent long-term therapeutic correction of phenylketonuria (PKU) in a mouse model by recombinant adeno-associated virus 8 pseudotyped vector-mediated gene transfer.
Volume: 13
Issue: 7
Pages: 587-93
Publication
First Author: McDonald JD
Year: 1990
Journal: Proc Natl Acad Sci U S A
Title: Pahhph-5: a mouse mutant deficient in phenylalanine hydroxylase.
Volume: 87
Issue: 5
Pages: 1965-7
Publication
First Author: Shedlovsky A
Year: 1993
Journal: Genetics
Title: Mouse models of human phenylketonuria.
Volume: 134
Issue: 4
Pages: 1205-10
Publication  
First Author: Bruinenberg VM
Year: 2016
Journal: Front Behav Neurosci
Title: The Behavioral Consequence of Phenylketonuria in Mice Depends on the Genetic Background.
Volume: 10
Pages: 233
Publication
First Author: Dyer CA
Year: 1996
Journal: J Neuropathol Exp Neurol
Title: Evidence for central nervous system glial cell plasticity in phenylketonuria.
Volume: 55
Issue: 7
Pages: 795-814
Publication
First Author: Mordhorst A
Year: 2021
Journal: FASEB J
Title: Phenylalanine hydroxylase contributes to serotonin synthesis in mice.
Volume: 35
Issue: 6
Pages: e21648
Publication
First Author: Christensen R
Year: 2005
Journal: Exp Dermatol
Title: Characterization of transgenic mice with the expression of phenylalanine hydroxylase and GTP cyclohydrolase I in the skin.
Volume: 14
Issue: 7
Pages: 535-42
Publication
First Author: Thöny B
Year: 2004
Journal: FEBS Lett
Title: Tetrahydrobiopterin protects phenylalanine hydroxylase activity in vivo: implications for tetrahydrobiopterin-responsive hyperphenylalaninemia.
Volume: 577
Issue: 3
Pages: 507-11
Publication
First Author: Hamman KJ
Year: 2011
Journal: Mol Genet Metab
Title: Hepatocytes from wild-type or heterozygous donors are equally effective in achieving successful therapeutic liver repopulation in murine phenylketonuria (PKU).
Volume: 104
Issue: 3
Pages: 235-40
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus musculus
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: McDonald JD
Year: 1997
Journal: Genomics
Title: Characterization of mutations at the mouse phenylalanine hydroxylase locus.
Volume: 39
Issue: 3
Pages: 402-5
Publication
First Author: Solverson P
Year: 2012
Journal: Am J Physiol Endocrinol Metab
Title: Glycomacropeptide, a low-phenylalanine protein isolated from cheese whey, supports growth and attenuates metabolic stress in the murine model of phenylketonuria.
Volume: 302
Issue: 7
Pages: E885-95
Publication
First Author: Sawin EA
Year: 2014
Journal: Mol Genet Metab
Title: Differential effects of low-phenylalanine protein sources on brain neurotransmitters and behavior in C57Bl/6-Pah(enu2) mice.
Volume: 111
Issue: 4
Pages: 452-61
Publication
First Author: Joseph B
Year: 2003
Journal: J Neurochem
Title: Relationship between myelin production and dopamine synthesis in the PKU mouse brain.
Volume: 86
Issue: 3
Pages: 615-26
Publication
First Author: Pascucci T
Year: 2013
Journal: PLoS One
Title: Behavioral and neurochemical characterization of new mouse model of hyperphenylalaninemia.
Volume: 8
Issue: 12
Pages: e84697
Publication
First Author: Ercal N
Year: 2002
Journal: Free Radic Biol Med
Title: Oxidative stress in a phenylketonuria animal model.
Volume: 32
Issue: 9
Pages: 906-11
Publication
First Author: Pascucci T
Year: 2002
Journal: Neuroreport
Title: Deficits in brain serotonin synthesis in a genetic mouse model of phenylketonuria.
Volume: 13
Issue: 18
Pages: 2561-4
Publication
First Author: Kornguth S
Year: 1994
Journal: Neuroimage
Title: Near-microscopic magnetic resonance imaging of the brains of phenylalanine hydroxylase-deficient mice, normal littermates, and of normal BALB/c mice at 9.4 Tesla.
Volume: 1
Issue: 3
Pages: 220-9
Publication
First Author: Dobrowolski SF
Year: 2014
Journal: Mol Genet Metab
Title: Methylome repatterning in a mouse model of Maternal PKU Syndrome.
Volume: 113
Issue: 3
Pages: 194-9
Publication
First Author: Lu L
Year: 2011
Journal: Mol Genet Metab
Title: Mechanisms regulating superoxide generation in experimental models of phenylketonuria: an essential role of NADPH oxidase.
Volume: 104
Issue: 3
Pages: 241-8
Publication
First Author: Bruinenberg VM
Year: 2019
Journal: PLoS One
Title: Long-term dietary intervention with low Phe and/or a specific nutrient combination improve certain aspects of brain functioning in phenylketonuria (PKU).
Volume: 14
Issue: 3
Pages: e0213391
Publication
First Author: Durrer KE
Year: 2017
Journal: PLoS One
Title: Genetically engineered probiotic for the treatment of phenylketonuria (PKU); assessment of a novel treatment in vitro and in the PAHenu2 mouse model of PKU.
Volume: 12
Issue: 5
Pages: e0176286
Publication
First Author: Seagraves NJ
Year: 2012
Journal: Mol Genet Metab
Title: Cardiac teratogenicity in mouse maternal phenylketonuria: defining phenotype parameters and genetic background influences.
Volume: 107
Issue: 4
Pages: 650-8
Publication  
First Author: Pascucci T
Year: 2008
Journal: Brain Res
Title: Reduced availability of brain amines during critical phases of postnatal development in a genetic mouse model of cognitive delay.
Volume: 1217
Pages: 232-8
Publication
First Author: Cho S
Year: 2001
Journal: Mol Genet Metab
Title: Effect of maternal blood phenylalanine level on mouse maternal phenylketonuria offspring.
Volume: 74
Issue: 4
Pages: 420-5
Publication
First Author: Ney DM
Year: 2008
Journal: J Nutr
Title: Dietary glycomacropeptide supports growth and reduces the concentrations of phenylalanine in plasma and brain in a murine model of phenylketonuria.
Volume: 138
Issue: 2
Pages: 316-22
Publication
First Author: Solverson P
Year: 2012
Journal: PLoS One
Title: Low bone strength is a manifestation of phenylketonuria in mice and is attenuated by a glycomacropeptide diet.
Volume: 7
Issue: 9
Pages: e45165
Publication
First Author: Gropper SS
Year: 2004
Journal: Mol Genet Metab
Title: Plasma phenylalanine concentrations are associated with hepatic iron content in a murine model for phenylketonuria.
Volume: 82
Issue: 1
Pages: 76-82
Publication
First Author: Smith CB
Year: 2000
Journal: Proc Natl Acad Sci U S A
Title: Cerebral protein synthesis in a genetic mouse model of phenylketonuria.
Volume: 97
Issue: 20
Pages: 11014-9
Publication
First Author: Sawin EA
Year: 2015
Journal: Am J Physiol Gastrointest Liver Physiol
Title: Glycomacropeptide is a prebiotic that reduces Desulfovibrio bacteria, increases cecal short-chain fatty acids, and is anti-inflammatory in mice.
Volume: 309
Issue: 7
Pages: G590-601
Publication
First Author: Goldfinger M
Year: 2017
Journal: Mol Genet Metab
Title: Partial rescue of neuropathology in the murine model of PKU following administration of recombinant phenylalanine ammonia lyase (pegvaliase).
Volume: 122
Issue: 1-2
Pages: 33-35
Publication
First Author: Puglisi-Allegra S
Year: 2000
Journal: Neuroreport
Title: Dramatic brain aminergic deficit in a genetic mouse model of phenylketonuria.
Volume: 11
Issue: 6
Pages: 1361-4
Publication
First Author: Crook N
Year: 2019
Journal: Cell Host Microbe
Title: Adaptive Strategies of the Candidate Probiotic E. coli Nissle in the Mammalian Gut.
Volume: 25
Issue: 4
Pages: 499-512.e8
Publication
First Author: Embury JE
Year: 2005
Journal: Pediatr Res
Title: Pathologic and immunohistochemical findings in hypothalamic and mesencephalic regions in the pah(enu2) mouse model for phenylketonuria.
Volume: 58
Issue: 2
Pages: 283-7