Objectives: To investigate the relationship between metabolic markers of cobalamin deficiency and cognitive function in normal older adults.
Design: Cross-sectional study.
Setting: Queen's University and St. Mary's of the Lake Hospital, Kingston, Ontario, Canada.
Participants: Two hundred eighty-one cognitively normal, community-dwelling participants aged 65 and older.
Measurements: Serum cobalamin, red blood cell folate, methylcitric acid, homocysteine, and methylmalonic acid were determined. Cognitive instruments included the California Verbal Learning Test, Mattis Dementia Rating Scale, and the Stroop Neuropsychological Screening Inventory (Stroop).
Results: Serum levels of methylcitric acid had a significant negative correlation with recall, learning, and discriminability (factor 1) of the California Verbal Learning Test after adjusting for age and sex (beta=-0.138, P=.019). Subjects with elevated methylcitric acid had significantly lower scores (factor 1) than subjects with normal methylcitric acid (P<.01). Bivariate analysis showed significant correlations between levels of homocysteine and the Stroop score and between cobalamin, methylmalonic acid, and homocysteine and some scores of the California Verbal Learning Test, but these relationships did not remain significant after multivariate analysis. Subjects with high homocysteine (tHcy) had lower Stroop scores than subjects with normal tHcy (P<.05). No biochemical parameters were associated with the Mattis Dementia Rating Scale scores.
Conclusion: This study indicates that, in normal elderly subjects, some cognitive scores are related to serum methylcitric acid and possibly homocysteine.
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http://dx.doi.org/10.1111/j.1532-5415.2004.52012.x | DOI Listing |
J Environ Manage
January 2025
School of Environmental Science and Engineering, Zhejiang Gongshang University, Hangzhou, 310018, PR China; International Science and Technology Cooperation Platform for Low-Carbon Recycling of Waste and Green Development, Zhejiang Gongshang University, Hangzhou, 310012, PR China. Electronic address:
Volatile fatty acids (VFAs) are suitable substrates for synthesizing poly(3-hydroxybutyrate-co-3-hydroxyvalerate) (PHBV), wherein propionate is a precursor of PHBV biosynthesis; however, high concentrations are toxic to bacteria. Therefore, VFAs with suitable ratio are needed. Here, with the ratio of acetate: propionate: butyrate being 1:4:2, the maximum PHBV content and the 3HV content were 46.
View Article and Find Full Text PDFFront Genet
November 2024
Department of Endocrinology, Genetics and Metabolism, Jiangxi Provincial Children's Hospital, Nanchang, Jiangxi, China.
Introduction: Holocarboxylase synthetase deficiency (HLCSD) is a rare autosomal recessive genetic disorder caused by mutations in the holocarboxylase synthetase (HLCS) gene, which affects multiple systems. Common clinical manifestations include metabolic acidosis, rash, feeding difficulties, and growth retardation, with predominant involvement of the nervous system, skin, and hair. However, respiratory symptoms as the initial manifestation are relatively rare.
View Article and Find Full Text PDFJ Chromatogr B Analyt Technol Biomed Life Sci
August 2024
Department of Genetics and Metabolism, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China. Electronic address:
Homocysteine, methionine, methylmalonic acid and 2-methylcitric acid are clinically relevant markers in the methionine, propionate, and cobalamin metabolism. This study aimed to develop and validate an ultra-performance liquid chromatography-tandem mass spectrometry (UPLC-MS/MS) method for simultaneously determining total homocysteine, methionine, methylmalonic acid and 2-methylcitric acid in dried blood spots. Three 3.
View Article and Find Full Text PDFInt J Neonatal Screen
July 2024
Department of Mother and Child, The Regional Center for Neonatal Screening, Diagnosis and Treatment of Inherited Congenital Metabolic and Endocrinological Diseases, AOUI Verona, 37134 Verona, Italy.
Methylmalonyl-CoA epimerase enzyme (MCEE) is responsible for catalyzing the isomeric conversion between D- and L-methylmalonyl-CoA, an intermediate along the conversion of propionyl-CoA to succinyl-CoA. A dedicated test for MCEE deficiency is not included in the newborn screening (NBS) panels but it can be incidentally identified when investigating methylmalonic acidemia and propionic acidemia. Here, we report for the first time the biochemical description of a case detected by NBS.
View Article and Find Full Text PDFFront Nutr
June 2024
Department of Pediatrics, Peking University First Hospital, Beijing, China.
Backgroud: Routine metabolic assessments for methylmalonic acidemia (MMA), propionic acidemia (PA), and homocysteinemia involve detecting metabolites in dried blood spots (DBS) and analyzing specific biomarkers in serum and urine. This study aimed to establish a liquid chromatography-tandem mass spectrometry (LC-MS/MS) method for the simultaneous detection of three specific biomarkers (methylmalonic acid, methylcitric acid, and homocysteine) in DBS, as well as to appraise the applicability of these three DBS metabolites in monitoring patients with MMA, PA, and homocysteinemia during follow-up.
Methods: A total of 140 healthy controls and 228 participants were enrolled, including 205 patients with MMA, 17 patients with PA, and 6 patients with homocysteinemia.
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