ArticleProceedings of the National Academy of Sciences of the United States of America1995
Fungal metabolic model for human type I hereditary tyrosinaemia.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1995. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 65 citations in OpenAlex.
- Reciprocal Genome Projection Reveals Reference-Conditioned Functional Variation in ClinicalPathogens (Basel, Switzerland) · 2026Article
- Unlocking Testosterone Production by Biotransformation: Engineering a Fungal Model ofBiomolecules · 2024Article
- TheFrontiers in cellular and infection microbiology · 2024Article
- Pyomelanin Synthesis inFrontiers in microbiology · 2021Article
- Intracellular growth is dependent on tyrosine catabolism in the dimorphic fungal pathogen Penicillium marneffei.PLoS pathogens · 2015Article
- Ecology drives the distribution of specialized tyrosine metabolism modules in fungi.Genome biology and evolution · 2014Article
- Disruption of fumarylacetoacetate hydrolase causes spontaneous cell death under short-day conditions in Arabidopsis.Plant physiology · 2013Article
- Role of the phenylalanine-hydroxylating system in aromatic substance degradation and lipid metabolism in the oleaginous fungus Mortierella alpina.Applied and environmental microbiology · 2013Article
- Ochronosis in a murine model of alkaptonuria is synonymous to that in the human condition.Osteoarthritis and cartilage · 2012Article
- Resolving phenylalanine metabolism sheds light on natural synthesis of penicillin G in Penicillium chrysogenum.Eukaryotic cell · 2012Article
- Proteomics shows new faces for the old penicillin producer Penicillium chrysogenum.Journal of biomedicine & biotechnology · 2012Review
- Genome and transcriptome analyses of the mountain pine beetle-fungal symbiont Grosmannia clavigera, a lodgepole pine pathogen.Proceedings of the National Academy of Sciences of the United States of America · 2011Article
- Pyomelanin formation in Aspergillus fumigatus requires HmgX and the transcriptional activator HmgR but is dispensable for virulence.PloS one · 2011Article
- skn-1-Dependent and -independent regulation of aip-1 expression following metabolic stress in Caenorhabditis elegans.Molecular and cellular biology · 2010Article
- Production of pyomelanin, a second type of melanin, via the tyrosine degradation pathway in Aspergillus fumigatus.Applied and environmental microbiology · 2009Article
- The Caenorhabditis elegans K10C2.4 gene encodes a member of the fumarylacetoacetate hydrolase family: a Caenorhabditis elegans model of type I tyrosinemia.The Journal of biological chemistry · 2008Article
- Novel phacB-encoded cytochrome P450 monooxygenase from Aspergillus nidulans with 3-hydroxyphenylacetate 6-hydroxylase and 3,4-dihydroxyphenylacetate 6-hydroxylase activities.Eukaryotic cell · 2007Article
- Fungal metabolic model for tyrosinemia type 3: molecular characterization of a gene encoding a 4-hydroxy-phenyl pyruvate dioxygenase from Aspergillus nidulans.Eukaryotic cell · 2006Article
- The homogentisate pathway: a central catabolic pathway involved in the degradation of L-phenylalanine, L-tyrosine, and 3-hydroxyphenylacetate in Pseudomonas putida.Journal of bacteriology · 2004Article
- The bacterial transposon Tn7 causes premature polyadenylation of mRNA in eukaryotic organisms: TAGKO mutagenesis in filamentous fungi.Nucleic acids research · 2003Article
Corrections and comments
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Type I hereditary tyrosinaemia (HT1) is a severe human inborn disease resulting from loss of fumaryl-acetoacetate hydrolase (Fah). Homozygous disruption of the gene encoding Fah in mice causes neonatal lethality, seriously limiting use of this animal as a model. We report here that fahA, the gene encoding Fah in the fungus Aspergillus nidulans, encodes a polypeptide showing 47.1% identity to its human homologue, fahA disruption results in secretion of succinylacetone (a diagnostic compound for human type I tyrosinaemia) and phenylalanine toxicity. We have isolated spontaneous suppressor mutations preventing this toxicity, presumably representing loss-of-function mutations in genes acting upstream of fahA in the phenylalanine catabolic pathway. Analysis of a class of these mutations demonstrates that loss of homogentisate dioxygenase (leading to alkaptonuria in humans) prevents the effects of a Fah deficiency. Our results strongly suggest human homogentisate dioxygenase as a target for HT1 therapy and illustrate the usefulness of this fungus as an alternative to animal models for certain aspects of human metabolic diseases.
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