| A | B |
|---|
| essential fatty acids that cross the bbb | linolenic and linoleic acid |
| synthesized in the brain; important in myelin formation | very long chain fatty acids |
| oxidized in peroxisomes by alpha-oxidation | VLC fatty acids and phytanic acid |
| affects peroxisome biogenesis | Refsum disease |
| synthesize 4x its own weight in lipids daily | oligodendrocytes |
| myelin basic proteins | help stabilize myelin structure from within the membrane |
| proteolipid protein protrudes into extracellular space | aids in compaction of the membranes via hydrophobic interactions |
| cerebrosides | 16% total myelin lipd |
| galactocerebrosides | pack tightly b/c lack charged group |
| CNS myelin proteins MBP PLP | PNS major myelin protein Po, P2 |
| antibodies directed against MBP | experimental allergic encephalomyelitis |
| predominant cerebroside galactosylcerebroside | single sugar attached to sphingosine |
| antigenic determinants of ABO bloodgroups | sphingolipids |
| ceraminde reacts with phosphatidylcholine | formation of sphingomyelin |
| ceramide reacts with UDP-sugars | formation of cerebrosides |
| sulfatides | sulfolipids of the brain |
| galatocerebroside reacts with PAPS- active sulfate donor) | formation of sulfatides |
| sphingolipidoses | lack of lysosomal enzymes to degrade sphingolipids |
| Ceramide-Glc-Gal | Globoside |
| Ceramide-Glc-Gal-GalNAc | Ganglioside |
| backbone of sphingolipids, derived from serine | sphingosine |
| glycolipids | derivatives of sphingosine |
| ceramide with carb groups attached to hydroxymethyl group | cerebrosides and gangliosides |
| Niemann-Pick disease | sphingomyelinase; cer:P-choline sphingomyelin |
| Gaucher disease | beta-glucosidase; Cer:Glc glucosylceramide |
| Krabbe disease | beta-galactosidase; Cer: Ga galactosylceramide |
| Tay-Sachs disease | Hexosaminidase A deficiency |
| oxidation of VLC fatty acids | generates acetyl CoA and NADH |
| step one of beta oxidation is catalyzed by | FAD-containing oxidase |
| Zellweger syndrome | defective peroxisomal biogenesis; liver and brain affected |
| elevation of C26:0 and C26:1 fatty acids in plasma | Zellwger, Refsum |
| Refsum disease | deficiency in phytanoly CoA hydroxylase |
| Refsum symptoms | retinitis pigmentosa, cerebellar ataxia, chronic polyneuropathy |
| cure Refsum | low phytanic acid diet |
| common branched chain fatty acids in diet | phytanic and pristanic acid ( from green veggies) |
| Tay-Sachs disease | accumulate GM2, not globoside |
| four base insertion in exon 11 of Hex A alpha-chain | Tay-Sachs premature stop codon |
