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Journal of Lipid Research, Vol. 44, 1737-1743, September 2003
Copyright © 2003 by American Society for Biochemistry and Molecular Biology

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

Keiko Tadano-Aritomi^*, Junko Matsuda, Hirokazu Fujimoto, Kunihiko Suzuki^** and Ineo Ishizuka^1,*

^* Department of Biochemistry, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo 173-8605, Japan
Department of Pediatrics, University of Tokushima School of Medicine, 3-18-15, Kuramoto-cho, Tokushima 770-8503, Japan
Mitsubishi Kagaku Institute of Life Sciences (MITILS), 11 Minamiooya, Machida, Tokyo 194-8511, Japan
^** Neuroscience Center, Departments of Neurology and Psychiatry, University of North Carolina School of Medicine, Chapel Hill, NC 27599

¹ To whom correspondence should be addressed. e-mail: ii{at}med.teikyo-u.ac.jp

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 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.

Supplementary key words spermatogenesis • electrospray ionization mass spectrometry • tandem mass spectrometry • liquid chromatography-mass spectrometry

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