{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,29]],"date-time":"2025-05-29T22:23:45Z","timestamp":1748557425559},"reference-count":24,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2008,8,19]],"date-time":"2008-08-19T00:00:00Z","timestamp":1219104000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Br J Nutr"],"abstract":"<jats:p>The aim of the present study was to examine the effects of cobalamin (Cbl) on the activity and expression of<jats:sc>l<\/jats:sc>-methylmalonyl-CoA mutase (MCM) in rat liver and cultured COS-7 cells. The MCM holoenzyme activity was less than 5\u00a0% of the total (holoenzyme+apoenzyme) activity in the liver although rats were fed a diet containing sufficient Cbl. When weanling rats were maintained on a Cbl-deficient diet, the holo-MCM activity became almost undetectable at the age of 10 weeks. In contrast, a marked increase in the total-MCM activity occurred under the Cbl-deficient conditions, and at the age of 20 weeks it was about 3-fold higher in the deficient rats than in the controls (108 (<jats:sc>sd<\/jats:sc>14\u00b75)<jats:italic>v.<\/jats:italic>35 (<jats:sc>sd<\/jats:sc>8\u00b75) nmol\/mg protein per min (<jats:italic>n<\/jats:italic>5);<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00\u00b705). Western blot analysis confirmed that the MCM protein level increased significantly in the Cbl-deficient rats. However, the MCM mRNA level, determined by real-time PCR, was rather decreased. When COS-7 cells were cultured in a medium in which 10\u00a0% fetal bovine serum was the sole source of Cbl, holo-MCM activity was barely detected. The supplementation of Cbl resulted in a large increase in the holo-MCM activity in the cells, but the activity did not exceed 30\u00a0% of the total-MCM activity even in the presence of Cbl at 10\u00a0\u03bcmol\/l. In contrast, the total-MCM activity was significantly decreased by the Cbl supplementation, indicating that Cbl deficiency results in an increase in the MCM protein level in COS-7 cells as well as in rat liver.<\/jats:p>","DOI":"10.1017\/s0007114508023398","type":"journal-article","created":{"date-parts":[[2008,8,19]],"date-time":"2008-08-19T08:43:31Z","timestamp":1219135411000},"page":"492-498","source":"Crossref","is-referenced-by-count":11,"title":["Cobalamin deficiency results in an abnormal increase in<scp>l<\/scp>-methylmalonyl-co-enzyme-A mutase expression in rat liver and COS-7 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