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Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria.
Published in:
Metabolites (2218-1989), 2022, v. 12, n. 10, p. 927, doi. 10.3390/metabo12100927
By:
- Norman, Brendan P.;
- Davison, Andrew S.;
- Hickton, Bryony;
- Ross, Gordon A.;
- Milan, Anna M.;
- Hughes, Andrew T.;
- Wilson, Peter J. M.;
- Sutherland, Hazel;
- Hughes, Juliette H.;
- Roberts, Norman B.;
- Bou-Gharios, George;
- Gallagher, James A.;
- Ranganath, Lakshminarayan R.
Publication type:
Article
Expression of tyrosine pathway enzymes in mice demonstrates that homogentisate 1,2‐dioxygenase deficiency in the liver is responsible for homogentisic acid‐derived ochronotic pigmentation.
Published in:
Journal of Inherited Metabolic Disease Reports, 2021, v. 58, n. 1, p. 52, doi. 10.1002/jmd2.12184
By:
- Wilson, Peter J. M.;
- Ranganath, Lakshminarayan R.;
- Bou‐Gharios, George;
- Gallagher, James A.;
- Hughes, Juliette H.
Publication type:
Article
Homogentisic acid is not only eliminated by glomerular filtration and tubular secretion but also produced in the kidney in alkaptonuria.
Published in:
Journal of Inherited Metabolic Disease, 2020, v. 43, n. 4, p. 737, doi. 10.1002/jimd.12181
By:
- Ranganath, Lakshminarayan R.;
- Milan, Anna M.;
- Hughes, Andrew T.;
- Khedr, Milad;
- Davison, Andrew S.;
- Shweihdi, Ella;
- Norman, Brendan P.;
- Hughes, Juliette H.;
- Bygott, Helen;
- Luangrath, Emily;
- Fitzgerald, Richard;
- Psarelli, Eftychia E.;
- Kan, Christa;
- Laan, Dinny;
- Olsson, Birgitta;
- Rudebeck, Mattias;
- Mankowitz, Louise;
- Sireau, Nicolas;
- Arnoux, Jean‐Baptiste;
- Le Quan Sang, Kim‐Hanh
Publication type:
Article
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.
Published in:
Journal of Inherited Metabolic Disease, 2020, v. 43, n. 2, p. 259, doi. 10.1002/jimd.12172
By:
- Hughes, Juliette H.;
- Wilson, Peter J. M.;
- Sutherland, Hazel;
- Judd, Shirley;
- Hughes, Andrew T.;
- Milan, Anna M.;
- Jarvis, Jonathan C.;
- Bou‐Gharios, George;
- Ranganath, Lakshminarayan R.;
- Gallagher, James A.
Publication type:
Article
Conditional targeting in mice reveals that hepatic homogentisate 1,2-dioxygenase activity is essential in reducing circulating homogentisic acid and for effective therapy in the genetic disease alkaptonuria.
Published in:
Human Molecular Genetics, 2019, v. 28, n. 23, p. 3928, doi. 10.1093/hmg/ddz234
By:
- Hughes, Juliette H.;
- Liu, Ke;
- Plagge, Antonius;
- Wilson, Peter J.M.;
- Sutherland, Hazel;
- Norman, Brendan P;
- Hughes, Andrew T.;
- Keenan, Craig M;
- Milan, Anna M.;
- Sakai, Takao;
- Ranganath, Lakshminarayan R.;
- Gallagher, James A.;
- Bou-Gharios, George
Publication type:
Article
Anatomical Distribution of Ochronotic Pigment in Alkaptonuric Mice is Associated with Calcified Cartilage Chondrocytes at Osteochondral Interfaces.
Published in:
Calcified Tissue International, 2021, v. 108, n. 2, p. 207, doi. 10.1007/s00223-020-00764-6
By:
- Hughes, Juliette H.;
- Keenan, Craig M.;
- Sutherland, Hazel;
- Edwards, Henry R.;
- Wilson, Peter J. M.;
- Ranganath, Lakshminarayan R.;
- Jarvis, Jonathan C.;
- Bou-Gharios, George;
- Gallagher, James A.
Publication type:
Article
Hereditary Tyrosinemia Type 1 Mice under Continuous Nitisinone Treatment Display Remnants of an Uncorrected Liver Disease Phenotype.
Published in:
Genes, 2023, v. 14, n. 3, p. 693, doi. 10.3390/genes14030693
By:
- Neuckermans, Jessie;
- Lequeue, Sien;
- Claes, Paul;
- Heymans, Anja;
- Hughes, Juliette H.;
- Colemonts-Vroninks, Haaike;
- Marcélis, Lionel;
- Casimir, Georges;
- Goyens, Philippe;
- Martens, Geert A.;
- Gallagher, James A.;
- Vanhaecke, Tamara;
- Bou-Gharios, George;
- De Kock, Joery
Publication type:
Article

Found: 7

Comprehensive Biotransformation Analysis of Phenylalanine-Tyrosine Metabolism Reveals Alternative Routes of Metabolite Clearance in Nitisinone-Treated Alkaptonuria.

Expression of tyrosine pathway enzymes in mice demonstrates that homogentisate 1,2‐dioxygenase deficiency in the liver is responsible for homogentisic acid‐derived ochronotic pigmentation.

Homogentisic acid is not only eliminated by glomerular filtration and tubular secretion but also produced in the kidney in alkaptonuria.

Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.

Conditional targeting in mice reveals that hepatic homogentisate 1,2-dioxygenase activity is essential in reducing circulating homogentisic acid and for effective therapy in the genetic disease alkaptonuria.

Anatomical Distribution of Ochronotic Pigment in Alkaptonuric Mice is Associated with Calcified Cartilage Chondrocytes at Osteochondral Interfaces.

Hereditary Tyrosinemia Type 1 Mice under Continuous Nitisinone Treatment Display Remnants of an Uncorrected Liver Disease Phenotype.