EC Number   |
General Information |
Reference |
|---|
   2.8.2.11 | malfunction |
CST knockout (CST-/-) mice, in which sulfatide is completely depleted, are born healthy, but display myelin abnormalities and progressive tremors starting at 4-6 weeks of age. A one-third reduction of the major compact-myelin proteins (myelin basic protein, MBP, and proteolipid protein, PLP) and an equivalent post-developmental loss of myelin lipids is detected, providing the molecular basis behind the thinner myelin sheaths. Lipidomics data demonstrate that the observed global reduction of myelin lipid content is not due to an increase of lipid degradation but rather to the reduction of their synthesis by oligodendrocytes. Sulfatide depletion leads to region-specific effects on non-compact myelin, dramatically affecting the paranode (neurofascin 155) and the major inner-tongue myelin protein (myelin-associated glycoprotein). Progressive decline of high molecular weight PLP complexes in CST-/- mice. Loss of ST results in reduced GalCer synthesis and/or increased GalCer degradation. Proposed molecular mechanisms underlying the effects of ST depletion on myelin structure/function, overview |
761717 |
| 2.8.2.11 | more |
analysis of the major CNS myelin proteins and the major lipids enriched in the myelin in a spatiotemporal manner, overview |
761717 |
| 2.8.2.11 | physiological function |
cerebroside sulfotransferase (CST) catalyzes the production of sulfatide, a major class of myelin-specific lipids. Sulfatide plays a critical role in myelin maintenance/function. Sulfatide (ST) promotes the interaction between adjacent PLP extracellular domains. The myelin sheath is a multilamellar, spirally wrapping extension of the plasma membrane of oligodendrocytes (OLs) in the central nervous system (CNS). Sulfatide mediates intraperiod line and myelin-axon junction interactions, and it plays a critical role in maintaining compact myelin, nodal and paranodal homeostasis |
761717 |
| 2.8.2.11 | physiological function |
CST is involved in sulfatide biosynthesis |
702073 |
| 2.8.2.11 | physiological function |
the enzyme is essential for spermatogenesis |
725110, 725957 |
