Article

Seminolipid and its precursor/degradative product, galactosylalkylacylglycerol, in the testis of saposin A- and prosaposin-deficient mice.

Department of Biochemistry, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo 173-8605, Japan.
The Journal of Lipid Research (impact factor: 5.56). 10/2003; 44(9):1737-43. DOI:10.1194/jlr.M300119-JLR200 pp.1737-43
Source: PubMed

ABSTRACT Sphingolipid activator proteins (saposins A, B, C, and D) are derived from a common precursor protein (prosaposin) and specifically activate in vivo degradation of glycolipids with short carbohydrate chains. A mouse model of prosaposin deficiency (prosaposin-/-) closely mimics the human disease with an elevation of multiple glycolipids. The recently developed saposin A-/- mice showed a chronic form of globoid cell leukodystrophy, establishing the essential in vivo role of saposin A as an activator for galactosylceramidase to degrade galactosylceramide. Seminolipid, the principal glycolipid in spermatozoa, and its precursor/degradative product, galactosylalkylacylglycerol (GalEAG), were analyzed in the testis of the two mouse mutants by electrospray ionization mass spectrometry. Saposin A-/- mice showed the normal seminolipid level, while that of prosaposin-/- mice was approximately 150% of the normal level at the terminal stage. In contrast, GalEAG increased up to 10 times in saposin A-/- mice, whereas it decreased with age in the wild-type as well as in prosaposin-/- mice. These analytical findings on the two saposin mutants may shed some light on the physiological function of seminolipid and GalEAG.

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    Article: The ether lipid-deficient mouse: tracking down plasmalogen functions.
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Keywords

chronic form
 
common precursor protein
 
degrade galactosylceramide
 
developed saposin A-/- mice
 
electrospray ionization mass spectrometry
 
globoid cell leukodystrophy
 
human disease
 
multiple glycolipids
 
normal level
 
normal seminolipid level
 
precursor/degradative product
 
principal glycolipid
 
prosaposin-/-
 
prosaposin-/- mice
 
saposin A-/- mice
 
short carbohydrate chains
 
Sphingolipid activator proteins
 
two mouse mutants
 
two saposin mutants
 
vivo degradation
 

Keiko Tadano-Aritomi