Background Remethylation defects are rare inherited disorders in which impaired
remethylation of homocysteine to methionine leads to accumulation of homocysteine and …

Met metabolism occurs primarily by activation of Met to AdoMet and further metabolism of
AdoMet by either the transmethylation-transsulfuration pathway or the polyamine …

The final unique stage in the metabolism of L-isoleucine involves the cleavage of 2-
methylacetoacetyl-CoA to acetyl-CoA and propionyl-CoA (Section 10.4). The propionyl-CoA …

Combined methylmalonic acidemia and homocystinuria, cblC type, is an inborn error of
intracellular cobalamin metabolism with a wide spectrum of clinical manifestations that is …

Cyanide is traditionally viewed as a cytotoxic agent, with its primary mode of action being the
inhibition of mitochondrial Complex IV (cytochrome c oxidase). However, recent studies …

The cblC defect is the most common inborn error of vitamin B12 metabolism. Despite
therapeutic measures, the long-term outcome is often unsatisfactory. This retrospective …

The clbC form of methylmalonic acidaemia is a rare and poorly understood condition which
results from impaired biosynthesis of methylcobalamin and adenosylcobalamin. The …

Background The cblC defect is a rare inborn error of intracellular cobalamin metabolism.
Biochemical hallmarks are elevated homocysteine and low methionine in plasma …

Abstract Cobalamin C (Cbl-C) defect is the most common inborn cobalamin metabolism
error; it causes impaired conversion of dietary vitamin B12 into its two metabolically active …

The increasing worldwide prevalence of dementia is a major public health concern. Findings
from some epidemiological studies suggest that diet and nutrition may be important …