Fatty acid synthesis from radiopropionate was evaluated in sural nerve biopsy slices from five normal controls and nine patients with pernicious anemia. The nerves were incubated in [14C]propionate, the lipids were extracted, and the fatty acid methyl esters were chromatographed by gas-liquid chromatography. In the normal nerves the radiolabel was found primarily in short chain (C12 and C14) fatty acids. The nerves from pernicious anemia patients showed two fatty acids peaks that were not discernible in the normal nerves, and these fatty acids had retention times intermediate to those of myristic (C14·0) and palmitic (C16·0) acids and palmitoleic (C16·1) and stearic (C18·0) acids, respectively. These two peaks (a C15 and C17 fatty acid) contained the bulk of the radioactivity recovered in the fatty acid fraction after incubation with [14C]propionate. Catalytic reduction and rechromatography failed to alter the retention time of these compounds suggesting that they are not unsaturated fatty acids. The nerves from the pernicious anemia patients had a decrease in the mean content of normal fatty acids when compared with the nerves from control patients as well as a decrease in the mean synthesis of normal fatty acids as estimated by isotope incorporation after incubation with [14C]propionate or 3H2O. Analysis of myelin isolated from the nerves indicated that the changes at least in part were in that fraction.
Eugene P. Frenkel
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Maternal vitamin B12 deficiency in rats alters DNA methylation in metabolically important genes in their offspring
VS Tanwar, S Ghosh, S Sati, S Ghose, L Kaur, KA Kumar, KV Shamsudheen, A Patowary, M Singh, V Jyothi, P Kommineni, S Sivasubbu, V Scaria, M Raghunath, R Mishra, GR Chandak, S Sengupta |
Molecular and Cellular Biochemistry | 2020 |
Vitamin B12 deficiency as a cause of severe neurological symptoms in breast fed infant – a case report
C Dubaj, K Czyż, W Furmaga-Jabłońska |
Italian Journal of Pediatrics | 2020 |
Vitamin B12: advances and insights
R Obeid |
2017 | |
Abnormal fatty acids in Canadian children with autism
J Jory |
Nutrition | 2016 |
Inborn Metabolic Diseases
JM Saudubray, MR Baumgartner, J Walter |
Inborn Metabolic Diseases | 2016 |
Handbook of Vitamins, Fifth Edition
R Rucker, K Bauerly |
Handbook of Vitamins, Fifth Edition | 2013 |
Vitamin-Responsive Epileptic Encephalopathies in Children
S Agadi, MM Quach, Z Haneef |
Epilepsy Research and Treatment | 2013 |
Nitrous oxide-induced B12 deficiency myelopathy: Perspectives on the clinical biochemistry of vitamin B12
L Hathout, S El-Saden |
Journal of the Neurological Sciences | 2011 |
Homocysteine, B Vitamins, and Cognitive Function
J Miller |
Diet Brain Behavior Practical Implications | 2011 |
Vitamins & Hormones
KJ Hare, FK Knop |
Vitamins & Hormones | 2010 |
Modern Nutrition
K LaPoint, M McIntosh |
Handbook of Nutrition in the Aged, Fourth Edition | 2008 |
Handbook of Vitamins, Fourth Edition
D Mock |
Handbook of Vitamins, Fourth Edition | 2007 |
Advances in Enzymology - and Related Areas of Molecular Biology
H Waelsch |
Advances in Enzymology and Related Areas of Molecular Biology | 2006 |
Coma and respiratory failure in a child with severe vitamin B12 deficiency
D Codazzi, F Sala, R Parini, M Langer |
Pediatric Critical Care Medicine | 2005 |
Severe encephalopathy with epilepsy in an infant caused by subclinical maternal pernicious anaemia: case report and review of the literature
GC Korenke, DH Hunneman, S Eber, F Hanefeld |
European Journal of Pediatrics | 2004 |
Chemistry and Biology of Pteridines and Folates
S Milstien, G Kapatos, RA Levine, B Shane |
2002 | |
Nutrition
&NA; |
Current Opinion in Lipidology | 1999 |
Persistence of neurological damage induced by dietary vitamin B-12 deficiency in infancy
U Schenck, C Bender-Götze, B Koletzko |
Archives of disease in childhood | 1997 |
Alternative Ernährung bei Kindern in der Kontroverse
B Koletzko |
1996 | |
6 Metabolite assays in cobalamin and folate deficiency
R Green |
Baillière's Clinical Haematology | 1995 |
Cobalt-vitamin B12 deficiency causes accumulation of odd- numbered, branched-chain fatty acids in the tissues of sheep
DG Kennedy, S Kennedy, WJ Blanchflower, JM Scott, DG Weir, AM Molloy, PB Young |
British Journal of Nutrition | 1994 |
Elevation of 2-methylcitric acid I and II levels in serum, urine, and cerebrospinal fluid of patients with cobalamin deficiency
RH Allen, SP Stabler, DG Savage, J Lindenbaum |
Metabolism | 1993 |
Folate–vitamin B12 interrelationships in the central nervous system
JM Scott |
Proceedings of the Nutrition Society | 1992 |
Biochemical Pharmacology of Blood and Bloodforming Organs
JW Fisher |
1992 | |
The Neurology of Cobalamin
MI Shevell, DS Rosenblatt |
CAN J NEUROL SCI | 1992 |
Methylmalonic acid metabolism and nervous-system fatty acids in cobalamin-deficient fruit bats receiving supplements of methionine, valine and isoleucine
E Vieira-Makings, N Chetty, SC Reavis, J Metz |
Biochemical Journal | 1991 |
Methylmalonyl-CoA mutase (EC 5.4.99.2) and methionine synthetase (EC 2.1.1.13) in the tissues of cobalt–vitamin B12 deficient sheep
DG Kennedy, A Cannavan, A Molloy, FO harte, SM Taylor, S Kennedy, WJ Blanchflower |
British Journal of Nutrition | 1990 |
Function of vitamin B12 in the central nervous system as revealed by congenital defects
CA Hall |
American Journal of Hematology | 1990 |
Elevation of total homocysteine in the serum of patients with cobalamin or folate deficiency detected by capillary gas chromatography-mass spectrometry
SP Stabler, PD Marcell, ER Podell, RH Allen, DG Savage, J Lindenbaum |
Journal of Clinical Investigation | 1988 |
Nutritional Modulation of Neural Function
WH Karasov |
Nutritional Modulation of Neural Function | 1988 |
Neuropsychiatric Disorders Caused by Cobalamin Deficiency in the Absence of Anemia or Macrocytosis
J Lindenbaum, EB Healton, DG Savage, JC Brust, TJ Garrett, ER Podell, PD Margell, SP Stabler, RH Allen |
New England Journal of Medicine | 1988 |
Sensory peripheral neuropathy of vitamin B12 deficiency: A primary demyelinating disease?
I Steiner, D Kidron, D Soffer, I Wirguin, O Abramsky |
Journal of Neurology | 1988 |
Failure to detect beta-leucine in human blood or leucine 2,3-aminomutase in rat liver using capillary gas chromatography-mass spectrometry
SP Stabler, J Lindenbaum, RH Allen |
The Journal of biological chemistry | 1988 |
Tissue folates in fruit bats (Rousettus aegyptiacus) with nitrous oxide-induced vitamin B12 deficiency and neurological impairment
JV der Westhuyzen, RE Davis, GC Icke, J Metz |
British Journal of Nutrition | 1987 |
Vitamin B12 and brain development
K Stollhoff, FJ Schulte |
European Journal of Pediatrics | 1987 |
The fruit bat as an experimental model of the neuropathy of cobalamin deficiency
J Metz, J der Westhuyzen |
Comparative Biochemistry and Physiology Part A: Physiology | 1987 |
Aktuelle Neuropädiatrie 1986
H Fichsel |
1987 | |
Vitamin B12
SM Innis |
Canadian Institute of Food Science and Technology Journal | 1986 |
Cobalamin inactivation decreases purine and methionine synthesis in cultured lymphoblasts
GR Boss |
Journal of Clinical Investigation | 1985 |
Disorders of cobalamin metabolism
Y Kano, S Sakamoto, Y Miura, F Takaku, V Herbert |
Critical Reviews in Oncology/Hematology | 1985 |
Selected Topics from Neurochemistry
RF Butterworth |
Selected Topics from Neurochemistry | 1985 |
Pathological Neurochemistry
A Lajtha |
1985 | |
The peripheral neuropathy of vitamin B12 deficiency
PA McCombe, JG McLeod |
Journal of the Neurological Sciences | 1984 |
Lipid Composition of the Brain in the Vitamin B12-Deficient Fruit Bat (Rousettus aegyptiacus) with Neurological Impairment
J Westhuyzen, RC Cantrill, F Fernandes-Costa, J Metz |
Journal of Neurochemistry | 1982 |
Fatty acid composition of synaptosomes from normal and cobalamin deficient bat brain
JV der Westhuyzen, G Lashansky, RC Cantrill |
Comparative Biochemistry and Physiology Part B: Comparative Biochemistry | 1982 |
Nitrous oxide has multiple deleterious effects on cobalamin metabolism and causes decreases in activities of both mammalian cobalamin-dependent enzymes in rats
H Kondo, ML Osborne, JF Kolhouse, MJ Binder, ER Podell, CS Utley, RS Abrams, RH Allen |
Journal of Clinical Investigation | 1981 |
Reversible Dementia and Neuropathy Associated with Folate Deficiency 16 Years After Partial Gastrectomy
C Enk, K Hougaard, E Hippe |
Scandinavian Journal of Haematology | 1981 |
Neurotoxicity of folates: Implications for vitamin B12 deficiency and Huntington's chorea
MJ Brennan, J der Westhuyzen, S Kramer, J Metz |
Medical Hypotheses | 1981 |
Vitamine
KH Bässler, K Lang |
1981 | |
Trace Element Metabolism in Man and Animals
JM Gawthorne, JM Howell, CL White |
1981 | |
INTERACTIONS OF THIAMIN, RIBOFLAVIN, AND OTHER B-VITAMINS
HE Sauberlich |
Annals of the New York Academy of Sciences | 1980 |
Optic atrophy in experimental vitamin B12 deficiency in monkeys
EM Chester, DP Agamanolis, JW Harris, M Victor, JD Hines, JA Kark |
Acta Neurologica Scandinavica | 1980 |
Amino acid transmitter transport in nerve endings from normal and vitamin B12 deficient fruit bats
MJ Brennan, RC Cantrill, SJ Warner, JV der Westhuyzen, F Fernandes-Costa, S Kramer, J Metz |
Brain Research | 1980 |
Biochemistry of Brain
N Colman, V Herbert |
Biochemistry of Brain | 1980 |
Nutrition and the Adult: Macronutrients Volume 3A
RB Alfin-Slater, D Kritchevsky |
1980 | |
Principles of Metabolic Control in Mammalian Systems
RH Herman, RM Cohn, PD McNamara |
1980 | |
Cobalamin-dependent formation of leucine and beta-leucine by rat and human tissue. Changes in pernicious anemia
JM Poston |
The Journal of biological chemistry | 1980 |
The Mechanism of Adenosylcobalamin-Dependent Reaction
BM Babior, JS Krouwer, HP Hogenkamp |
Critical Reviews in Biochemistry and Molecular Biology | 1979 |
Enzymatic properties of mitochondria isolated from normal and vitamin B12-deficient rats
MA Matlib, EP Frenkel, A Mukherjee, J Henslee, PA Srere |
Archives of Biochemistry and Biophysics | 1979 |
Effects of vitamin B12 deficiency on lipid metabolism of the rat liver and nervous system
C Fehling, M JÄgerstad, B Åkesson, J Axelsson, A Brun |
British Journal of Nutrition | 1978 |
EFFECT OF 1-AMINOCYCLOPENTANE-1-CARBOXYLIC ACID (CYCLOLEUCINE) ON DEVELOPING RAT CENTRAL NERVOUS SYSTEM PHOSPHOLIPIDS
RB Ramsey, VW Fischer |
Journal of Neurochemistry | 1978 |
Studies on the absorption of calcium, phosphorus, magnesium, copper and zinc by sheep fed on roughage-cereal diets
MH Stevenson, EF Unsworth |
British Journal of Nutrition | 1978 |
Newer Aspects of Pernicious Anemia
L Kass |
Critical Reviews in Clinical Laboratory Sciences | 1978 |
Fatty acid composition of myelin isolated from the brain of a patient with cellular deficiency of co-enzyme forms of vitamin B12
RB Ramsey, T Scott, NL Banik |
Journal of the Neurological Sciences | 1977 |
HLA phenotype A2;B12 in vitamin B12 neuromyelopathy
MA Horton, RT Oliver |
Journal of the Neurological Sciences | 1977 |
Methylmalonic acidemia controlled with oral administration of vitamin B12
BA Gordon, RA Carson |
Canadian Medical Association journal | 1976 |
Effect of methylmalonate on ketone body metabolism in developing rat brain
MS Patel, OE Owen, C Raefsky |
Life Sciences | 1976 |
Vitamin B12—Folate Interrelations
KC Das, V Herbert |
Clinics in Haematology | 1976 |
Neurological Aspects of Folate and Vitamin B12 Metabolism
EH Reynolds |
Clinics in Haematology | 1976 |
Biochemical and ultrastructural hepatic changes during vitamin B12 deficiency in animals and man
EP Frenkel, A Mukherjee, CR Hackenbrock, PA Srere |
The Journal of biological chemistry | 1976 |
Intracellular Localization of Hepatic Propionyl-CoA Carboxylase and Methylmalonyl-CoA Mutase in Humans and Normal and Vitamin B12Deficient Rats
EP Frenkel, RL Kitchens |
British Journal of Haematology | 1975 |
Effect of vitamin B-12 deficiency on the hepatic tissue concentration of acyl carnitines
WC Burton, EP Frenkel |
Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism | 1975 |
Characterization of branched chain fatty acids from subcutaneous triacyglycerols of barley-fed lambs
WR Duncan, AK Lough, GA Garton, P Brooks |
Lipids | 1974 |
Effect of Vitamin B12 Deprivation on the in Vivo Levels of Coenzyme A Intermediates Associated with Propionate Metabolism
EP Frenkel, RL Kitchens, LB Hersh, R Frenkel |
The Journal of biological chemistry | 1974 |
The Effect of Vitamin B12 Deprivation on the Enzymes of Fatty Acid Synthesis
EP Frenkel, RL Kitchens, JM Johnston |
The Journal of biological chemistry | 1973 |