Identification of cytidine diphosphodiesters in erythrocytes from a patient with pyrimidine nucleotidase deficiency

M. S. Swanson, Rodney Smith Markin, S. J. Stohs, C. R. Angle

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20 Citations (Scopus)

Abstract

Pyrimidine 5'-nucleotidase deficient (PND) erythrocytes contain elevated levels of pyrimidine nucleotides and relatively normal purine nucleotide levels. The composition of this nucleotide pool has been examined by others, but not all of the abnormal red cell metabolites in this disorder were identified. We have isolated and positively confirmed the identity of cytidine diphosphate (CDP)-choline and CDP-ethanolamine from PND red cells using methods including proton FT-NMR, spectroscopy, and comparative mass spectrometry. The concentrations of these and other pyrimidine nucleotidase-deficient erythrocyte nucleotides were determined using anion-exchange high performance liquid chromatography and ultraviolet (u.v.) detection. The pyrimidine diphosphodiesters appear to be the most prominent abnormal pyrimidine nucleotides in PND red cells, accounting for 55% of the total red cell pyrimidine nucleotides in this disorder. It is proposed that these abnormal phosphodiesters may be related to the accelerated hemolysis in PND.

Original languageEnglish (US)
Pages (from-to)665-670
Number of pages6
JournalBlood
Volume63
Issue number3
StatePublished - Jan 1 1984

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nucleotidase
Cytidine
5'-Nucleotidase
Pyrimidine Nucleotides
Erythrocytes
Cells
Nucleotides
Cytidine Diphosphate Choline
Purine Nucleotides
High performance liquid chromatography
Metabolites
Hemolysis
Nuclear magnetic resonance spectroscopy
Anions
Mass spectrometry
Protons
Mass Spectrometry
Magnetic Resonance Spectroscopy
High Pressure Liquid Chromatography
pyrimidine

ASJC Scopus subject areas

  • Hematology

Cite this

Identification of cytidine diphosphodiesters in erythrocytes from a patient with pyrimidine nucleotidase deficiency. / Swanson, M. S.; Markin, Rodney Smith; Stohs, S. J.; Angle, C. R.

In: Blood, Vol. 63, No. 3, 01.01.1984, p. 665-670.

Research output: Contribution to journalArticle

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