Document Detail

Methylmalonic acid metabolism and nervous-system fatty acids in cobalamin-deficient fruit bats receiving supplements of methionine, valine and isoleucine.
MedLine Citation:
PMID:  1674859     Owner:  NLM     Status:  MEDLINE    
Cobalamin neuropathy was produced in Cape fruit bats (Rousettus aegyptiacus) by a cobalamin-free diet combined with intermittent exposure to nitrous oxide, which inactivates cobalamin. Cobalamin-deficient bats had low hepatic methylmalonyl-CoA mutase holoenzyme activity, with elevated plasma and urinary methylmalonic acid levels. No significant changes could be demonstrated in the concentration of odd- or branched-chain fatty acids in the nervous system. Supplementation of the cobalamin-free diet with methionine, valine or isoleucine delayed the onset of neuropathy, despite persistence of methylmalonic acid accumulation. Supplementation with any of the three amino acids was associated with elevation of hepatic methionine concentration. The action of valine and isoleucine in delaying the onset of neuropathy can be explained by their methionine-sparing effect. These results emphasize the central role of methionine in the cobalamin neuropathy and do not support the hypothesis that the neuropathy is related to an effect of cobalamin deficiency on the propionic acid pathway.
E Vieira-Makings; N Chetty; S C Reavis; J Metz
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Biochemical journal     Volume:  275 ( Pt 3)     ISSN:  0264-6021     ISO Abbreviation:  Biochem. J.     Publication Date:  1991 May 
Date Detail:
Created Date:  1991-07-03     Completed Date:  1991-07-03     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  2984726R     Medline TA:  Biochem J     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  585-90     Citation Subset:  IM    
Department of Haematology, School of Pathology, South African Institute for Medical Research, Johannesburg.
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MeSH Terms
Amino Acids / administration & dosage,  pharmacology*
Brain / metabolism
Chiroptera / metabolism*
Fatty Acids / metabolism*
Isoleucine / administration & dosage,  pharmacology
Liver / enzymology
Methionine / administration & dosage,  metabolism,  pharmacology
Methylmalonic Acid / metabolism*
Methylmalonyl-CoA Mutase / metabolism
Nervous System / metabolism*
Nervous System Diseases / etiology,  metabolism
Spinal Cord / metabolism
Valine / administration & dosage,  pharmacology
Vitamin B 12 Deficiency / complications,  metabolism*
Reg. No./Substance:
0/Amino Acids; 0/Fatty Acids; 516-05-2/Methylmalonic Acid; 63-68-3/Methionine; 7004-03-7/Valine; 73-32-5/Isoleucine; EC Mutase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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