Lipid abnormalities in hereditary neuropathy. Part 2. Serum phospholipids.
The mean percentage of linoleate to total fatty acids in phosphatidylcholine and lysophosphatidylcholine fractions of serum phospholipids from neuropathic patients with HMN (hereditary motor neuropathy, also called distal type of progressive muscular atrophy), HMSN-I and HMSN-II (two types of peroneal muscular atrophy), and FA (Friedreich's ataxia) was reduced by approximately 10--20% (P less than 0.001). On the other hand, the mean percentage of nervonic acid in sphingomyelin was elevated by 9--20%. No significant difference was observed in phosphatidylethanolamine between neuropathic patients and control subjects. The mean concentration of phosphatidylcholine and sphingomyelin was also significantly reduced in neuropathic patients (except in HMN and HMSN-III). A significant correlation between endogenous 2-linoleoyl-sn-glycerol-3-phosphocholine and cholesteryl linoleate synthesis in vitro suggests that the decreased activity of phosphatidylcholine acyltransferase (EC 2.3.1.43; LCAT) in neuropathic patients is influenced by the fatty acid composition of their lipoprotein substrate. Furthermore, the reduction of phosphatidylcholine and of cholesteryl linoleate synthesis in vitro in neuropathic patients was affected by age and sex. It is unlikely that the reduced linoleate level in serum phosphatidylcholine for most, possibly all, of the inherited neuropathies studied here reflects a specific biochemical disorder. Possibly it reflects a more generalized biochemical alteration common to inherited neuropathy. One possibility is that biosynthesis of new membrane in axonal regeneration, segmental remyelination and Schwann cell hyperplasia may reduce the serum linoleate pool.
D000328:Adult; D000367:Age Factors; D055598:Chemical Phenomena; D002621:Chemistry; D002788:Cholesterol Esters / Q000097:blood; D005227:Fatty Acids; D005260:Female; D005621:Friedreich Ataxia / Q000097:blood* / Q000201:enzymology; D006801:Humans; D008244:Lysophosphatidylcholines / Q000097:blood; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000097:blood / Q000201:enzymology / Q000235:genetics*; D007862:Phosphatidylcholine-Sterol O-Acyltransferase / Q000378:metabolism; D010713:Phosphatidylcholines / Q000097:blood; D010714:Phosphatidylethanolamines / Q000097:blood; D010743:Phospholipids / Q000097:blood*; D012737:Sex Factors; D013109:Sphingomyelins / Q000097:blood; D013577:Syndrome
D002788:Cholesterol Esters; D005227:Fatty Acids; D008244:Lysophosphatidylcholines; D010713:Phosphatidylcholines; D010714:Phosphatidylethanolamines; D010743:Phospholipids; D013109:Sphingomyelins; D007862:Phosphatidylcholine-Sterol O-Acyltransferase