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Literature summary for 2.4.2.4 extracted from

  • Lopez, L.C.; Akman, H.O.; Garcia-Cazorla, A.; Dorado, B.; Marti, R.; Nishino, I.; Tadesse, S.; Pizzorno, G.; Shungu, D.; Bonilla, E.; Tanji, K.; Hirano, M.
    Unbalanced deoxynucleotide pools cause mitochondrial DNA instability in thymidine phosphorylase-deficient mice (2009), Hum. Mol. Genet., 18, 714-722.
    View publication on PubMedView publication on EuropePMC

Protein Variants

Protein Variants Comment Organism
additional information generation of thymidine phosphorylase, TP, and uridine phosphorylase, UP, EC 2.4.2.3, double knockout mice, TP-/-UP-/- by insertion mutagenesis, which show severe TP deficiency, increased thymidine and deoxyuridine levels in tissues and elevated mitochondrial deoxythymidine triphosphate. As consequences of the nucleotide pool imbalances, brains of mutant mice developed partial depletion of mtDNA, deficiencies of respiratory chain complexes and encephalopathy, accounting for the pathogenesis of mitochondrial neurogastrointestinal encephalopathy, overview. Mitochondria respiratory chain deficiency in the brain of TP-/-UP-/- mice, overview Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
thymidine + phosphate Mus musculus
-
thymine + 2-deoxy-alpha-D-ribose 1-phosphate
-
r
thymidine + phosphate Mus musculus C57Bl6/J
-
thymine + 2-deoxy-alpha-D-ribose 1-phosphate
-
r

Organism

Organism UniProt Comment Textmining
Mus musculus
-
C57Bl6/J mice
-
Mus musculus C57Bl6/J
-
C57Bl6/J mice
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mus musculus
-
heart
-
Mus musculus
-
kidney
-
Mus musculus
-
liver
-
Mus musculus
-
lung
-
Mus musculus
-
muscle
-
Mus musculus
-
small intestine
-
Mus musculus
-
spleen
-
Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
no activities in other tissues than liver from TP-/-UP-/- mice Mus musculus
0.00002
-
wild-type mouse muscle Mus musculus
0.000205
-
wild-type mouse heart Mus musculus
0.000243
-
TP-/-UP-/- mouse liver Mus musculus
0.000285
-
wild-type mouse spleen Mus musculus
0.00067
-
wild-type mouse kidney Mus musculus
0.000862
-
wild-type mouse lung Mus musculus
0.00136
-
wild-type mouse brain Mus musculus
0.0014
-
wild-type mouse liver Mus musculus
0.00946
-
wild-type mouse small intestine Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
thymidine + phosphate
-
Mus musculus thymine + 2-deoxy-alpha-D-ribose 1-phosphate
-
r
thymidine + phosphate
-
Mus musculus C57Bl6/J thymine + 2-deoxy-alpha-D-ribose 1-phosphate
-
r