Study of transaldolase deficiency in urine samples by capillary LC-MS/MS.
J Mass Spectrom
; 41(4): 463-9, 2006 Apr.
Article
em En
| MEDLINE
| ID: mdl-16470722
ABSTRACT
Transaldolase (TAL) is a key enzyme of the pentose phosphate pathway (PPP). TAL deficiency is a newly recognized cause of liver cirrhosis. We have developed an ion-pair LC separation combined with negative ion electrospray MS/MS detection method to assess PPP metabolites in urine samples from TAL-deficient mice. Sedoheptulose 7-phosphate (S7P), C5-polyols D-arabitol and D-ribitol, and 6-phosphogluconate (6PG) levels were markedly increased in urine of TAL-deficient mice with respect to those of wild-type and heterozygote littermates. The detection limits of S7P, D-arabitol, and 6PG were 0.15 +/- 0.015 pmol, 3.5 +/- 0.41 pmol, and 0.61 +/- 0.055 pmol, respectively. The limit of quantitation was 0.4 +/- 0.024 nmol/ml for S7P, 1.6 +/- 0.11 nmol/ml for 6PG and 10 +/- 0.7 nmol/ml for D-arabitol. Additional metabolites, hexose 6-phosphates (m/z 259), D-ribose 5-phosphate and D-xylulose 5-phosphate (m/z 229), D-fructose 1,6-diphosphate (m/z 339), C6-polyols (m/z 181) and GSSG (m/z 611), that have been positively identified in mouse urine, showed similar levels in control and TAL-deficient mice.
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Via de Pentose Fosfato
/
Espectrometria de Massas
/
Fosfatos Açúcares
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Transaldolase
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Urinálise
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Eletrocromatografia Capilar
Limite:
Animals
Idioma:
En
Ano de publicação:
2006
Tipo de documento:
Article