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Literature summary for 3.1.3.7 extracted from

  • Hudson, B.H.; Frederick, J.P.; Drake, L.Y.; Megosh, L.C.; Irving, R.P.; York, J.D.
    Role for cytoplasmic nucleotide hydrolysis in hepatic function and protein synthesis (2013), Proc. Natl. Acad. Sci. USA, 110, 5040-5045.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine mice deficient for bisphosphate 3'-nucleotidase Bpnt1 do not exhibit skeletal defects but instead develop severe liver pathologies, including hypoproteinemia, hepatocellular damage, and in severe cases, frank whole body edema and death. These phenotypes are accompanied by tissue-specific elevations of the substrate 3?-phosphoadenosine 5'-phosphate, up to 50fold in liver, repressed translation, and aberrant nucleolar architecture. The phenotypes of the Bpnt1 knockout are rescued by generating a double mutant mouse deficient for both 3'-phosphoadenosine 5'-phosphate synthesis and hydrolysis, consistent with a mechanism in which 3'-phosphoadenosine 5'-phosphate accumulation is toxic to tissue function independent of sulfation Mus musculus

Organism

Organism UniProt Comment Textmining
Mus musculus Q9Z0S1
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-

Synonyms

Synonyms Comment Organism
bisphosphate 3'-nucleotidase 1
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Mus musculus
BPNT1
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Mus musculus

General Information

General Information Comment Organism
physiological function mice deficient for bisphosphate 3'-nucleotidase Bpnt1 do not exhibit skeletal defects but instead develop severe liver pathologies, including hypoproteinemia, hepatocellular damage, and in severe cases, frank whole body edema and death. These phenotypes are accompanied by tissue-specific elevations of the substrate 3'-phosphoadenosine 5'-phosphate, up to 50fold in liver, repressed translation, and aberrant nucleolar architecture. The phenotypes of the Bpnt1 knockout are rescued by generating a double mutant mouse deficient for both 3'-phosphoadenosine 5'-phosphate synthesis and hydrolysis, consistent with a mechanism in which 3'-phosphoadenosine 5'-phosphate accumulation is toxic to tissue function independent of sulfation Mus musculus