Alpha 2.0
Login Register
» Articles » PMID: 6708747

Lipids of Liver, Kidney, Spleen and Muscle in a Case of Generalized Deficiency of Cytochrome B5 Reductase in Congenital Methemoglobinemia with Mental Retardation

Overview
Journal Lipids
Specialty Biochemistry
Date 1984 Jan 1
PMID 6708747
Citations 5
Authors
H HIRONO
Affiliations
Soon will be listed here.
Abstract

The lipid compositions of liver, kidney, spleen and muscle in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation were analyzed and compared with age-matched controls. The contents of cholesterol and phospholipids (mumol/g) in each organ were within the normal range. Diminished proportions of unsaturated fatty acids were observed in ethanolamine phosphoglycerides (EPG) of the liver. The proportion of linoleic acid decreased to less than half of the normal level in EPG of liver, kidney and spleen.

Citing Articles

Cellular and Molecular Mechanisms of Recessive Hereditary Methaemoglobinaemia Type II.

Siendones E, Ballesteros M, Navas P J Clin Med. 2018; 7(10).

PMID: 30309019 PMC: 6210646. DOI: 10.3390/jcm7100341.

Electron Transfer Pathways in Cholesterol Synthesis.

Porter T Lipids. 2015; 50(10):927-36.

PMID: 26344922 DOI: 10.1007/s11745-015-4065-1.

Ncb5or deficiency increases fatty acid catabolism and oxidative stress.

Xu M, Wang W, Frontera J, Neely M, Lu J, Aires D J Biol Chem. 2011; 286(13):11141-54.

PMID: 21300801 PMC: 3064168. DOI: 10.1074/jbc.M110.196543.

Loss of Ncb5or results in impaired fatty acid desaturation, lipoatrophy, and diabetes.

Larade K, Jiang Z, Zhang Y, Wang W, Bonner-Weir S, Zhu H J Biol Chem. 2008; 283(43):29285-91.

PMID: 18682384 PMC: 2570878. DOI: 10.1074/jbc.M804645200.

Dietary linoleic acid and the fatty acid profiles in rats fed partially hydrogenated marine oils.

Hoy C, Holmer G Lipids. 1988; 23(10):973-80.

PMID: 3200121 DOI: 10.1007/BF02536346.

References
1.
HIRONO H . Lipids of myelin, white matter and gray matter in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. Lipids. 1980; 15(4):272-5. DOI: 10.1007/BF02535839. View
2.
Okayasu T, Ono T, Shinojima K . Involvement of cytochrome b5 in the oxidative desaturation of linoleic acid to gamma-linolenic acid in rat liver microsomes. Lipids. 1977; 12(3):267-71. DOI: 10.1007/BF02533345. View
3.
Bruce A . Skeletal muscle lipids. 3. Changes in fatty acid composition of individual phosphoglycerides in man from fetal to middle age. J Lipid Res. 1974; 15(2):109-13. View
4.
HIRONO H . Adipose fatty acid composition in a case of generalized deficiency of cytochrome b5 reductase in congenital methemoglobinemia with mental retardation. Tohoku J Exp Med. 1983; 140(4):391-4. DOI: 10.1620/tjem.140.391. View
5.
Lee T, Baker R, Stephens N, Snyder F . Evidence for participation of cytochrome b5 in microsomal delta-6 desaturation of fatty acids. Biochim Biophys Acta. 1977; 489(1):25-31. DOI: 10.1016/0005-2760(77)90228-4. View