Basically three lipid coatings are used for coating the CHITIns, which include (a) (PC) phosphatidylcholine to prepare PCCHITIns (b) (3L) with the mixed lipid composition, phosphatidylcholine: cholesterol (Chol): galactocerebroside
(GalC) to prepare (3LCHITIns), and (c) (4L) with the mixed lipid composition, phosphatidylcholine: cholesterol: galactocerebroside
: phosphatidylethanolamine (PE) to prepare (4LCHITIns).
Lysosomal enzyme testing showed galactocerebroside
beta galactosidase or galactocerebrosidase (GALC) level which was almost undetectable and definitely much below the 99th percentile for age and sex confirming the diagnosis of Krabbe disease.
KineMed's platforms will be utilised to measure the synthesis of certain myelin lipids and protein components, such as cholesterol, galactocerebroside
and phospholipids, as well as major myelin proteins, during periods of demyelination and remyelination in animal models.
2] Nonstandard abbreviations: PKU, phenylketonuria; BIA, bacterial inhibition assay; MS/MS, tandem mass spectrometry; LSD, lysosomal storage disorder; MPS II, mucopolysaccharidosis type II; HSCT, hematopoietic stem cell transplantation; CNS, central nervous system; ASM, acid sphingomyelinase deficiency; GAA, acid alpha glucosidase; ESI, electrospray ionization; GALC, galactocerebroside
Specifically, the straw-shaped microtubules, measuring some 120 nanometers in diameter, are made of the sugar-based lipid galactocerebroside
A In order for full-blown autoimmune demyelination to take place in guinea pigs, we think that two components of the myelin sheath must be seen by the immune system: myelin basic protein (MBP), which is the major causal antigen in the animal disease, and a lipid in the sheath called galactocerebroside
, or GC.
An adaptation of this method enabled direct analysis of galactocerebroside
[beta]-galactosidase activity in DBS for the diagnosis of Krabbe disease (18).
The Einstein group theorized that injecting a preparation containing two components of myelin -- myelin basic protein and galactocerebroside
-- might elicit a myelin-restoring immune response in guinea pigs with already established disease.
6] Nonstandard abbreviations: DBS, dried blood spot; ABC, acid [beta]-glucocerebrosidase; GLA, acid [alpha]-galactosidase A; GAA, lysosomal acid [alpha]-glucosidase; ASM, acid sphingomyelinase; GALC, galactocerebroside
[beta]-galactosidase; PMN, polymorphonuclear leukocyte; CID, collision-induced dissociation; and RAAG, renal acid [alpha]-glucosidase.
We investigated whether MS/MS can be used to directly assay enzymes in dried blood spots, in this case, galactocerebroside
[beta]-galactosidase (GALC; EC 3.
2]N, BN, C, cystine-derived, galactocerebroside
, metal, dichalcogenide, Mo[S.