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Tytuł :
Next-generation probiotics as a therapeutic strategy for the treatment of phenylketonuria: a review.
Autorzy :
de Oliveira Filho JG; Goiano Federal Institute of Education, Science, and Technology, Rio Verde, Goiás, Brazil.; School of Pharmaceutical Sciences, São Paulo State University (UNESP), Araraquara, São Paulo, Brazil.
Carvalho ASES; Goiano Federal Institute of Education, Science, and Technology, Rio Verde, Goiás, Brazil.
Alves JDS; Goiano Federal Institute of Education, Science, and Technology, Rio Verde, Goiás Brazil.
Egea MB; Goiano Federal Institute of Education, Science, and Technology, Rio Verde, Goiás, Brazil.
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Źródło :
Nutrition reviews [Nutr Rev] 2022 Sep 05; Vol. 80 (10), pp. 2100-2112.
Typ publikacji :
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylalanine Hydroxylase*/metabolism
Phenylalanine Hydroxylase*/therapeutic use
Phenylketonurias*/drug therapy
Phenylketonurias*/genetics
Probiotics*/therapeutic use
Adult ; Diet ; Humans ; Phenylalanine/metabolism ; Phenylalanine/therapeutic use
Czasopismo naukowe
Tytuł :
Thermodynamics of iron, tetrahydrobiopterin, and phenylalanine binding to phenylalanine hydroxylase from Chromobacterium violaceum.
Autorzy :
Li M; Department of Chemistry, Mississippi State University, Mississippi State, MS, 39762, USA.
Subedi BP; Department of Biochemistry, University of Texas Health Science Center, San Antonio, TX, 78229, USA.
Fitzpatrick PF; Department of Biochemistry, University of Texas Health Science Center, San Antonio, TX, 78229, USA. Electronic address: .
Emerson JP; Department of Chemistry, Mississippi State University, Mississippi State, MS, 39762, USA. Electronic address: .
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Źródło :
Archives of biochemistry and biophysics [Arch Biochem Biophys] 2022 Oct 30; Vol. 729, pp. 109378. Date of Electronic Publication: 2022 Aug 20.
Typ publikacji :
Journal Article
MeSH Terms :
Metalloproteins*/metabolism
Phenylalanine Hydroxylase*/chemistry
Phenylalanine Hydroxylase*/metabolism
Biopterin/analogs & derivatives ; Chromobacterium ; Ferrous Compounds ; Iron/metabolism ; Kinetics ; Phenylalanine/metabolism ; Pterins/chemistry ; Pterins/metabolism ; Thermodynamics ; Tyrosine
SCR Organism :
Chromobacterium violaceum
Czasopismo naukowe
Tytuł :
Fatal systemic disorder caused by biallelic variants in FARSA.
Autorzy :
Kim SY; Department of Genomic Medicine, Rare Disease Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro Jongno-gu, Seoul, 110-744, Korea.
Ko S; School of Biological Sciences, Seoul National University, Seoul, Korea.
Kang H; School of Biological Sciences, Seoul National University, Seoul, Korea.
Kim MJ; Department of Genomic Medicine, Rare Disease Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro Jongno-gu, Seoul, 110-744, Korea.
Moon J; Department of Genomic Medicine, Rare Disease Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro Jongno-gu, Seoul, 110-744, Korea.
Lim BC; Department Pediatrics, Pediatric Neuroscience Center, Seoul National University, Seoul, Korea.
Kim KJ; Department Pediatrics, Pediatric Neuroscience Center, Seoul National University, Seoul, Korea.
Choi M; Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Korea.
Choi HJ; School of Biological Sciences, Seoul National University, Seoul, Korea. .
Chae JH; Department of Genomic Medicine, Rare Disease Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro Jongno-gu, Seoul, 110-744, Korea. .; Department Pediatrics, Pediatric Neuroscience Center, Seoul National University, Seoul, Korea. .
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Źródło :
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2022 Aug 02; Vol. 17 (1), pp. 306. Date of Electronic Publication: 2022 Aug 02.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine-tRNA Ligase*/chemistry
Phenylalanine-tRNA Ligase*/genetics
Humans ; Infant, Newborn ; Mutation/genetics ; Phenylalanine ; RNA, Transfer/genetics ; Whole Exome Sequencing
Czasopismo naukowe
Tytuł :
Deubiquitinase USP19 extends the residual enzymatic activity of phenylalanine hydroxylase variants.
Autorzy :
Sarodaya N; Graduate School of Biomedical Science and Engineering, Hanyang University, Seoul, South Korea.
Tyagi A; Graduate School of Biomedical Science and Engineering, Hanyang University, Seoul, South Korea.
Kim HJ; Department of Pharmacology, College of Medicine, Hanyang University, Seoul, South Korea.
Kang JS; Department of Pharmacology, College of Medicine, Hanyang University, Seoul, South Korea.
Singh V; Department of Biosciences, School of Science, Indrashil University, Rajpur, Mehsana, Gujarat, India.
Hong SH; Department of Internal Medicine, School of Medicine, Kangwon National University, Chuncheon, South Korea.
Kim WJ; Department of Internal Medicine and Environmental Health Center, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon, South Korea.
Kim KS; Graduate School of Biomedical Science and Engineering, Hanyang University, Seoul, South Korea. .; College of Medicine, Hanyang University, Seoul, South Korea. .
Ramakrishna S; Graduate School of Biomedical Science and Engineering, Hanyang University, Seoul, South Korea. .; College of Medicine, Hanyang University, Seoul, South Korea. .
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Źródło :
Scientific reports [Sci Rep] 2022 Aug 20; Vol. 12 (1), pp. 14243. Date of Electronic Publication: 2022 Aug 20.
Typ publikacji :
Journal Article
MeSH Terms :
Deubiquitinating Enzymes*/genetics
Endopeptidases*/genetics
Phenylalanine Hydroxylase*/genetics
Phenylketonurias*/genetics
Animals ; Asians/genetics ; Disease Progression ; Humans ; Mammals ; Mutation ; Phenylalanine/genetics
Czasopismo naukowe
Tytuł :
Genetic etiology and clinical challenges of phenylketonuria.
Autorzy :
Elhawary NA; Department of Medical Genetics, College of Medicine, Umm Al-Qura University, P.O. Box 57543, Mecca, 21955, Saudi Arabia. .
AlJahdali IA; Department of Community Medicine, College of Medicine, Umm Al-Qura University, P.O. Box 57543, Mecca, 21955, Saudi Arabia.
Abumansour IS; Department of Medical Genetics, College of Medicine, Umm Al-Qura University, P.O. Box 57543, Mecca, 21955, Saudi Arabia.
Elhawary EN; Faculty of Medicine, MS Genomic Medicine Program, University of Southampton, Southampton General Hospital, Southampton, UK.
Gaboon N; Department of Clinical Genetics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Dandini M; Department of Laboratory and Blood Bank, Maternity and Children Hospital, Mecca, Saudi Arabia.
Madkhali A; Department of Pathology and Laboratory Medicine, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
Alosaimi W; Department of Hematology, Maternity and Children Hospital, Mecca, Saudi Arabia.
Alzahrani A; Department of Laboratory and Blood Bank at Maternity and Children Hospital, Mecca, Saudi Arabia.
Aljohani F; Department of Pediatric Clinics, Maternity and Children Hospital, King Salman Medical City, Madinah, Saudi Arabia.
Melibary EM; Department of Medical Genetics, College of Medicine, Umm Al-Qura University, P.O. Box 57543, Mecca, 21955, Saudi Arabia.
Kensara OA; Department of Clinical Nutrition, Faculty of Applied Medical Sciences, Umm Al-Qura University, Jeddah, Saudi Arabia.; Department of Biochemistry, Batterjee Medical College, Jeddah, Saudi Arabia.
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Źródło :
Human genomics [Hum Genomics] 2022 Jul 19; Vol. 16 (1), pp. 22. Date of Electronic Publication: 2022 Jul 19.
Typ publikacji :
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylalanine Hydroxylase*/metabolism
Phenylalanine Hydroxylase*/therapeutic use
Phenylketonurias*/genetics
Phenylketonurias*/therapy
Humans ; Phenylalanine/metabolism ; Phenylalanine/therapeutic use
Czasopismo naukowe
Tytuł :
Modeling the cognitive effects of diet discontinuation in adults with phenylketonuria (PKU) using pegvaliase therapy in PAH-deficient mice.
Autorzy :
Winn SR; Department of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Dudley S; Department of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Scherer T; Department of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Rimann N; Department of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Thöny B; Department of Pediatrics, University of Zurich, Steinwiessstrasse 75, Zurich CH-8032, Switzerland.
Boutros S; Department of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Krenik D; Department of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Raber J; Department of Behavioral Neuroscience, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA; Departments of Neurology and Radiation Medicine, Division of Neuroscience, ONPRC, Oregon Health & Science University, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA.
Harding CO; Department of Medical and Molecular Genetics, Oregon Health & Science University, Mailstop L-103, 3181 Sam Jackson Park Rd., Portland, OR 97239, USA. Electronic address: .
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Źródło :
Molecular genetics and metabolism [Mol Genet Metab] 2022 May; Vol. 136 (1), pp. 46-64. Date of Electronic Publication: 2022 Mar 21.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylketonurias*/drug therapy
Adult ; Animals ; Cognition ; Diet ; Disease Models, Animal ; Female ; Humans ; Mice ; Mice, Inbred C57BL ; Phenylalanine ; Phenylalanine Ammonia-Lyase ; Recombinant Proteins
Czasopismo naukowe
Tytuł :
The first step into phenolic metabolism in the hornwort Anthoceros agrestis: molecular and biochemical characterization of two phenylalanine ammonia-lyase isoforms.
Autorzy :
Pezeshki S; Institut für Pharmazeutische Biologie und Biotechnologie, Philipps-Universität Marburg, Robert-Koch-Str. 4, 35037, Marburg, Germany.
Warmbier I; Institut für Pharmazeutische Biologie und Biotechnologie, Philipps-Universität Marburg, Robert-Koch-Str. 4, 35037, Marburg, Germany.
Busch T; Institut für Pharmazeutische Biologie und Biotechnologie, Philipps-Universität Marburg, Robert-Koch-Str. 4, 35037, Marburg, Germany.
Bauerbach E; Institut für Pharmazeutische Biologie und Biotechnologie, Philipps-Universität Marburg, Robert-Koch-Str. 4, 35037, Marburg, Germany.
Szövenyi P; Institut für Systematische und Evolutionäre Botanik, Universität Zürich, Zollikerstrasse 107, 8008, Zurich, Switzerland.
Petersen M; Institut für Pharmazeutische Biologie und Biotechnologie, Philipps-Universität Marburg, Robert-Koch-Str. 4, 35037, Marburg, Germany. .
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Źródło :
Planta [Planta] 2022 Jul 07; Vol. 256 (2), pp. 33. Date of Electronic Publication: 2022 Jul 07.
Typ publikacji :
Journal Article
MeSH Terms :
Anthocerotophyta*/metabolism
Phenylalanine Ammonia-Lyase*/genetics
Phenylalanine Ammonia-Lyase*/metabolism
Histidine ; Phenols ; Phenylalanine ; Phylogeny ; Protein Isoforms/genetics ; Tyrosine
Czasopismo naukowe
Tytuł :
Molecular cloning and characterization of three phenylalanine ammonia-lyase genes from Schisandra chinensis.
Autorzy :
Fan SP; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Chen W; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Wei JC; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Gao XX; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Yang YC; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Wang AH; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China.
Hu GS; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China. Electronic address: hugsh_.
Jia JM; School of Traditional Chinese Materia Medica, Shenyang Pharmaceutical University, Shenyang 110016, China. Electronic address: .
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Źródło :
Chinese journal of natural medicines [Chin J Nat Med] 2022 Jul; Vol. 20 (7), pp. 527-536.
Typ publikacji :
Journal Article
MeSH Terms :
Phenylalanine Ammonia-Lyase*/chemistry
Schisandra*/genetics
Cloning, Molecular ; Escherichia coli/genetics ; Escherichia coli/metabolism ; Phenylalanine/genetics ; Phenylalanine/metabolism ; Recombinant Proteins
Czasopismo naukowe
Tytuł :
Preliminary Investigation of Microbiome and Dietary Differences in Patients with Phenylketonuria on Enzyme Substitution Therapy Compared to Traditional Therapies.
Autorzy :
McWhorter N; Department of Pediatrics, Division of Genetics and Metabolism, University of Kentucky, Lexington, Kentucky. Electronic address: .
Dhillon J; Department of Nutrition and Exercise Physiology, School of Medicine, University of Missouri, Columbia, Missouri.
Hoffman J; Department of Human Nutrition, College of Arts & Sciences, Winthrop University, Rock Hill, South Carolina.
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Źródło :
Journal of the Academy of Nutrition and Dietetics [J Acad Nutr Diet] 2022 Jul; Vol. 122 (7), pp. 1283-1295.e3. Date of Electronic Publication: 2021 Dec 28.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Diet*
Enzyme Replacement Therapy*
Gastrointestinal Microbiome*
Phenylalanine Ammonia-Lyase*/therapeutic use
Phenylketonurias*/therapy
Adult ; Cross-Sectional Studies ; Humans ; Phenylalanine ; Phenylalanine Hydroxylase ; Recombinant Proteins/therapeutic use
Czasopismo naukowe
Tytuł :
Preformulation Studies with Phenylalanine Ammonia Lyase: Essential Prelude to a Microcapsule Formulation for the Management of Phenylketonuria.
Autorzy :
Besada C; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Hakami A; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Pillai G; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Yetsko K; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Truong N; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Little T; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Pantano S; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States.
Dmello A; Department of Pharmaceutical Sciences, University of the Sciences in Philadelphia, Philadelphia, PA, United States. Electronic address: .
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Źródło :
Journal of pharmaceutical sciences [J Pharm Sci] 2022 Jul; Vol. 111 (7), pp. 1857-1867. Date of Electronic Publication: 2022 Mar 26.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Ammonia-Lyase*/metabolism
Phenylketonurias*/drug therapy
Animals ; Capsules ; Dipeptides ; Mice ; Phenylalanine/metabolism ; Serum Albumin, Bovine
Czasopismo naukowe
Tytuł :
Phenylketonuria oxidative stress and energy dysregulation: Emerging pathophysiological elements provide interventional opportunity.
Autorzy :
Dobrowolski SF; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA 15224, United States of America. Electronic address: .
Phua YL; Division of Medical Genetics, Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Vockley J; Division of Medical Genetics, Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Goetzman E; Division of Medical Genetics, Children's Hospital of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Blair HC; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA 15224, United States of America; Veteran's Affairs Medical Center, Pittsburgh, PA, United States of America.
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Źródło :
Molecular genetics and metabolism [Mol Genet Metab] 2022 Jun; Vol. 136 (2), pp. 111-117. Date of Electronic Publication: 2022 Mar 29.
Typ publikacji :
Journal Article; Review
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylketonurias*/genetics
Adult ; Glutathione Disulfide/metabolism ; Glutathione Reductase/metabolism ; Humans ; Oxidative Stress ; Phenylalanine ; Pyruvates ; Tyrosine/metabolism
Czasopismo naukowe
Tytuł :
Comparative metabolomics in the Pah classical PKU mouse identifies cerebral energy pathway disruption and oxidative stress.
Autorzy :
Dobrowolski SF; Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America. Electronic address: .
Phua YL; Division of Medical Genetics, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Sudano C; Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Spridik K; Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Zinn PO; Department of Neurological Surgery, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Wang Y; Division of Medical Genetics, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Bharathi S; Division of Medical Genetics, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Vockley J; Division of Medical Genetics, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
Goetzman E; Division of Medical Genetics, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15224, United States of America.
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Źródło :
Molecular genetics and metabolism [Mol Genet Metab] 2022 May; Vol. 136 (1), pp. 38-45. Date of Electronic Publication: 2022 Mar 18.
Typ publikacji :
Journal Article
MeSH Terms :
Phenylalanine Hydroxylase*
Phenylketonurias*/genetics
Animals ; Disease Models, Animal ; Humans ; Metabolomics ; Mice ; Oxidative Stress ; Phenylalanine ; Reactive Oxygen Species
Czasopismo naukowe
Tytuł :
α-Phenylalanyl tRNA synthetase competes with Notch signaling through its N-terminal domain.
Autorzy :
Ho MT; Institute of Cell Biology, University of Bern, Bern, Switzerland.
Lu J; Institute of Cell Biology, University of Bern, Bern, Switzerland.
Vazquez-Pianzola P; Institute of Cell Biology, University of Bern, Bern, Switzerland.
Suter B; Institute of Cell Biology, University of Bern, Bern, Switzerland.
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Źródło :
PLoS genetics [PLoS Genet] 2022 Apr 29; Vol. 18 (4), pp. e1010185. Date of Electronic Publication: 2022 Apr 29 (Print Publication: 2022).
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine-tRNA Ligase*/chemistry
Phenylalanine-tRNA Ligase*/genetics
Phenylalanine-tRNA Ligase*/metabolism
Animals ; Drosophila/genetics ; Phenylalanine ; RNA, Transfer, Phe/chemistry ; RNA, Transfer, Phe/metabolism
Czasopismo naukowe
Tytuł :
Alchemical Design of Pharmacological Chaperones with Higher Affinity for Phenylalanine Hydroxylase.
Autorzy :
Conde-Giménez M; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.
Galano-Frutos JJ; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.
Galiana-Cameo M; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.
Mahía A; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.
Victor BL; Coimbra Chemistry Center-Institute of Molecular Sciences (CQC-IMS), Department of Chemistry, University of Coimbra, 3004-535 Coimbra, Portugal.
Salillas S; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.
Velázquez-Campoy A; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.; Aragon Health Research Institute (IIS Aragón), 50009 Zaragoza, Spain.; CIBER de Enfermedades Hepáticas y Digestivas CIBERehd, Instituto de Salud Carlos III, 28029 Madrid, Spain.
Brito RMM; Coimbra Chemistry Center-Institute of Molecular Sciences (CQC-IMS), Department of Chemistry, University of Coimbra, 3004-535 Coimbra, Portugal.
Gálvez JA; Instituto de Síntesis Química y Catálisis Homogénea (ISQCH), Departamento de Química Orgánica, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.
Díaz-de-Villegas MD; Instituto de Síntesis Química y Catálisis Homogénea (ISQCH), Departamento de Química Orgánica, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.
Sancho J; Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, 50009 Zaragoza, Spain.; Biocomputation and Complex Systems Physics Institute (BIFI)-GBsC-CSIC Joint Unit, Universidad de Zaragoza, 50018 Zaragoza, Spain.; Aragon Health Research Institute (IIS Aragón), 50009 Zaragoza, Spain.
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Źródło :
International journal of molecular sciences [Int J Mol Sci] 2022 Apr 19; Vol. 23 (9). Date of Electronic Publication: 2022 Apr 19.
Typ publikacji :
Journal Article
MeSH Terms :
Phenylalanine Hydroxylase*/chemistry
Phenylketonurias*/metabolism
Calorimetry ; Humans ; Phenylalanine/metabolism ; Protein Folding
Czasopismo naukowe
Tytuł :
Gene expression profiles in the brain of phenylketonuria mouse model reversed by the low phenylalanine diet therapy.
Autorzy :
Hong S; Department of Neonatal Medicine, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Zhu T; Department of Neonatal Medicine, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Zheng S; Department of Neonatal Medicine, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Zhan X; Department of Pediatric Endocrinology and Genetic Metabolism, Shanghai Institute for Pediatric Research, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Kongjiang Road 1665#, Shanghai, 200092, China.
Xu F; Department of Pediatric Endocrinology and Genetic Metabolism, Shanghai Institute for Pediatric Research, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Kongjiang Road 1665#, Shanghai, 200092, China.
Gu X; Department of Pediatric Endocrinology and Genetic Metabolism, Shanghai Institute for Pediatric Research, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Kongjiang Road 1665#, Shanghai, 200092, China. .
Liang L; Department of Pediatric Endocrinology and Genetic Metabolism, Shanghai Institute for Pediatric Research, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Kongjiang Road 1665#, Shanghai, 200092, China. .
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Źródło :
Metabolic brain disease [Metab Brain Dis] 2021 Dec; Vol. 36 (8), pp. 2405-2414. Date of Electronic Publication: 2021 Sep 15.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylketonurias*/metabolism
Animals ; Brain/metabolism ; Disease Models, Animal ; Mice ; Phenylalanine/genetics ; Transcriptome
Czasopismo naukowe
Tytuł :
Evaluation of patients with phenylalanine metabolism disorder: a single center experience.
Autorzy :
Erdol S; Department of Pediatrics, Division of Metabolism, Uludag University Faculty of Medicine, Bursa, Turkey.
Bilgin H; Department of Pediatrics, Division of Metabolism, Uludag University Faculty of Medicine, Bursa, Turkey.
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Źródło :
Journal of pediatric endocrinology & metabolism : JPEM [J Pediatr Endocrinol Metab] 2022 Jan 27; Vol. 35 (4), pp. 463-467. Date of Electronic Publication: 2022 Jan 27 (Print Publication: 2022).
Typ publikacji :
Journal Article
MeSH Terms :
Phenylalanine Hydroxylase*/genetics
Phenylketonurias*
Biopterin ; Child ; Humans ; Phenylalanine ; Retrospective Studies
Czasopismo naukowe
Tytuł :
A New View of Bone Loss in Phenylketonuria.
Autorzy :
Dobrowolski SF; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA.
Tourkova IL; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA.; Pittsburgh Veteran's Affairs Medical Center, Pittsburgh, PA, USA.
Sudano CR; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA.
Larrouture QC; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA.; Pittsburgh Veteran's Affairs Medical Center, Pittsburgh, PA, USA.
Blair HC; Department of Pathology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA.; Pittsburgh Veteran's Affairs Medical Center, Pittsburgh, PA, USA.
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Źródło :
Organogenesis [Organogenesis] 2021 Oct 02; Vol. 17 (3-4), pp. 50-55. Date of Electronic Publication: 2021 Aug 25.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
MeSH Terms :
Bone Diseases, Metabolic*/etiology
Phenylalanine Hydroxylase*/deficiency
Phenylketonurias*/complications
Phenylketonurias*/drug therapy
Animals ; Disease Models, Animal ; Humans ; Mice ; Phenylalanine
Czasopismo naukowe
Tytuł :
Long-term efficacy and safety of sapropterin in patients who initiated sapropterin at < 4 years of age with phenylketonuria: results of the 3-year extension of the SPARK open-label, multicentre, randomised phase IIIb trial.
Autorzy :
Muntau AC; University Children's Hospital, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246, Hamburg, Germany. .
Burlina A; University Hospital, Padua, Italy.
Eyskens F; Universitair Ziekenhuis Antwerpen, Antwerp, Belgium.
Freisinger P; Children's Hospital Kreiskliniken, Reutlingen, Germany.
Leuzzi V; Universita La Sapienza, Rome, Italy.
Sivri HS; Hacettepe University School of Medicine, Ankara, Turkey.
Gramer G; Division for Neuropaediatrics and Metabolic Medicine, Centre for Paediatric and Adolescent Medicine, University Hospital Heidelberg, Heidelberg, Germany.
Pazdírková R; Department of Children and Adolescents, Third Faculty of Medicine, Charles University, Prague, Czech Republic.
Cleary M; Great Ormond Street Hospital, London, UK.
Lotz-Havla AS; Dr. Von Hauner Children's Hospital, Munich, Germany.
Lane P; BioMarin Europe Ltd., London, UK.
Alvarez I; BioMarin Europe Ltd., London, UK.
Rutsch F; Muenster University Children's Hospital, Muenster, Germany.
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Źródło :
Orphanet journal of rare diseases [Orphanet J Rare Dis] 2021 Aug 03; Vol. 16 (1), pp. 341. Date of Electronic Publication: 2021 Aug 03.
Typ publikacji :
Clinical Trial, Phase III; Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, Non-U.S. Gov't
MeSH Terms :
Phenylalanine Hydroxylase*
Phenylketonurias*/drug therapy
Biopterin/analogs & derivatives ; Biopterin/therapeutic use ; Child ; Child, Preschool ; Humans ; Phenylalanine
Czasopismo naukowe
Tytuł :
A whiff of the future: functions of phenylalanine-derived aroma compounds and advances in their industrial production.
Autorzy :
Skaliter O; Institute of Plant Sciences and Genetics in Agriculture, The Robert H. Smith Faculty of Agriculture, Food and Environment, The Hebrew University of Jerusalem, Rehovot, Israel.
Livneh Y; Institute of Plant Sciences and Genetics in Agriculture, The Robert H. Smith Faculty of Agriculture, Food and Environment, The Hebrew University of Jerusalem, Rehovot, Israel.
Agron S; Department of Neurobiology, The Weizmann Institute of Science, Rehovot, Israel.
Shafir S; B. Triwaks Bee Research Center, Department of Entomology, Institute of Environmental Sciences, Robert H. Smith Faculty of Agriculture, Food and Environment, The Hebrew University of Jerusalem, Rehovot, Israel.
Vainstein A; Institute of Plant Sciences and Genetics in Agriculture, The Robert H. Smith Faculty of Agriculture, Food and Environment, The Hebrew University of Jerusalem, Rehovot, Israel.
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Źródło :
Plant biotechnology journal [Plant Biotechnol J] 2022 Sep; Vol. 20 (9), pp. 1651-1669. Date of Electronic Publication: 2022 Jun 30.
Typ publikacji :
Journal Article; Review; Research Support, Non-U.S. Gov't
MeSH Terms :
Odorants*
Phenylalanine*/metabolism
Humans ; Metabolic Engineering ; Plants/genetics
Czasopismo naukowe
Tytuł :
Side-chain halogen effects on self-assembly and hydrogelation of cationic phenylalanine derivatives.
Autorzy :
Abraham BL; Department of Chemistry, University of Rochester, Rochester, NY 14627, USA. .
Mensah SG; Department of Chemistry, University of Rochester, Rochester, NY 14627, USA. .
Gwinnell BR; Department of Chemistry, University of Rochester, Rochester, NY 14627, USA. .
Nilsson BL; Department of Chemistry, University of Rochester, Rochester, NY 14627, USA. .; Materials Science Program, University of Rochester, Rochester, NY 14627, USA.
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Źródło :
Soft matter [Soft Matter] 2022 Aug 17; Vol. 18 (32), pp. 5999-6008. Date of Electronic Publication: 2022 Aug 17.
Typ publikacji :
Journal Article
MeSH Terms :
Halogens*
Phenylalanine*/analogs & derivatives
Phenylalanine*/chemistry
Cations ; Hydrogels/chemistry ; Molecular Weight
Czasopismo naukowe

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