RGD Reference Report - Tissue distribution, intracellular localization and proteolytic processing of rat 4-hydroxyphenylpyruvate dioxygenase. - Rat Genome Database

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Tissue distribution, intracellular localization and proteolytic processing of rat 4-hydroxyphenylpyruvate dioxygenase.

Authors: Neve, S  Aarenstrup, L  Tornehave, D  Rahbek-Nielsen, H  Corydon, TJ  Roepstorff, P  Kristiansen, K 
Citation: Neve S, etal., Cell Biol Int 2003;27(8):611-24.
RGD ID: 632936
Pubmed: PMID:12867153   (View Abstract at PubMed)

4-hydroxyphenylpyruvate dioxygenase (HPD) is an important enzyme involved in tyrosine catabolism. HPD was shown to be identical to a protein named the F-antigen, exploited by immunologists because of its unique immunological properties. Congenital HPD deficiency is a rare, relatively benign condition known as hereditary type III tyrosinemia. Decreased expression of HPD is often observed in association with the severe type I tyrosinemia, and interestingly, inhibition of HPD activity seems to ameliorate the clinical symptoms of type I tyrosinemia. In this study we present a comprehensive analysis of tissue specific expression and intracellular localization of HPD in the rat. By combined use of in situ hybridization and immunohistochemistry we confirm previously known sites of expression in liver and kidney. In addition, we show that HPD is abundantly expressed in neurons in the cortex, cerebellum and hippocampus. By using immunoelectron microscopy and confocal laser scanning microscopy, we provide evidence that HPD contrary to earlier assumptions specifically localizes to membranes of the endoplasmic reticulum and the Golgi apparatus. Detailed mass spectrometric analyses of HPD purified from rat liver revealed N-terminal and C-terminal processing of HPD, and expression of recombinant HPD suggested that C-terminal processing enhances the enzymatic activity.

Gene Ontology Annotations    Click to see Annotation Detail View

Biological Process
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
tyrosine catabolic process  TAS 632936 RGD 

Cellular Component
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
endoplasmic reticulum located_inIDA 632936PMID:12867153MGI 
endoplasmic reticulum membrane  IDA 632936 RGD 
Golgi apparatus located_inIDA 632936PMID:12867153MGI 
Golgi membrane  IDA 632936 RGD 

Molecular Function
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
4-hydroxyphenylpyruvate dioxygenase activity enablesIDA 632936PMID:12867153MGI 
4-hydroxyphenylpyruvate dioxygenase activity  TAS 632936 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Hpd  (4-hydroxyphenylpyruvate dioxygenase)


Additional Information