A Survey of Neurological Mutant Mice

Abstract

The lipids of white matter and peripheral nerve from neurological mutant mice with possible myelin abnormalities were analyzed by thin-layer chromatography and quantitated by densitometry. Eight mutants had major abnormalities in the central nervous system (CNS) and/or peripheral nervous system (PNS) tissues examined (optic nerve, and trigeminal and sciatic nerves). In the optic nerve of ax^J/ax^J, there were increases of 20–30% in the levels of the major phospholipids; peripheral nerve was normal. In bc^3J/bc^3JCNS, the major phospholipids and cholesterol were increased by 25–40%; the PNS was normal. In myd/myd CNS, there were increases of about 20% in the levels of both forms of cerebrosides and in the major phospholipids; in the PNS the lipids were normal, ot/ot CNS had 20–40% reductions of all the glycolipids and minor alterations in some of the phospholipids and cholesterol; the PNS had 20% losses of both forms of cerebrosides. In the PNS of ji/ji, there were decreases of 10–40% among the glycolipids and of 15–25% in three of the major phospholipids; the CNS was virtually normal. In the PNS of dt^J/dt^J, vb/vb and wr/wr, almost all lipids were significantly decreased. The CNS of dt^J/dt^Jand vb/vb were normal; wr/wr had minor reductions of certain glycolipids and phospholipids. Six mutants had relatively minor lipid abnormalities in their myelinated tissues. In cr/cr PNS, there were elevated levels of the cerebrosides and major phospholipids; the CNS was virtually normal. In db/db CNS and PNS, there were reduced levels of the nonhydroxy forms of cerebroside and sulfatide. The major change in htr/htr was the elevation of all the glycolipids in the CNS. In the CNS of Lc/+, nonhydroxy cerebroside was reduced. In shm/shm PNS, nonhydroxy sulfatide was elevated and there were small decreases in some of the phospholipids, wl/wl CNS showed decreases among most of the glycolipids. Mutants homozygous for du, mto, spa and tg had virtually normal lipid levels in both the optic and peripheral nerves.

Tellurium Blocks Cholesterol Synthesis by Inhibiting Squalene Metabolism: Preferential Vulnerability to This Metabolic Block Leads to Peripheral Nervous System Demyelination

“…System 2 was also used for the densitometric analysis of squalene because it provided the best resolution of squalene from other lipids. For a better resolution of cholesterol for densitometric and radioactive quantitative analyses, we used system 4, which involved consecutive development in two different solvent systems: chloroform/methanol/acetic acid (93.1 : 1.9: 0.1 by volume), which was developed to within 2 cm of the top, followed by hexaneldiethy1 ether/acetic acid (89.35.7: 0.1 by volume), in which the Rf for cholesterol is 0.4 (Ganser et al, 1988). The in vitro enzyme assay for squalene epoxidase, which required separation of squalene from sterols in the nonsaponifiable fraction, involved system 5: benzene/ acetone (90: 1 vol/vol).…”

“…Each TLC plate used for densitometric analysis contained four control or four experimental samples and five standards of either cholesterol or squalene. Plates were sprayed with a solution of 10% copper sulfate in 8% phosphoric acid (Ganser et al, 1988), and lipids were charred by heating for 4 min on an aluminum block preheated to 160°C in a Fisher Isotemp Oven. Charred TLC plates were then covered with a clear glass plate to minimize the time-dependent loss of color intensity.…”

Lipid-free versus lipid-bound P2 protein-induced experimental allergic neuritis: Clinicopathological, neurophysiological, and immunological study

“…Phospholipid content was determined as described by Chen et al (1956). TLC was carried out on Whatman LHPK silica gel 60 Å plates for HPTLC as in Ganser et al (1988). Lipids were extracted as described by Folch et al (1957) from samples containing 30 g protein and detected by 10% CuSO 4 in 8% H 3 PO 4 .…”

Myelin Abnormalities in the Optic and Sciatic Nerves in Mice With GM1-Gangliosidosis

“…Primary myelination is completed early in development; however, the amount of myelin in humans and rodents continues to increase with age in the central nervous system (CNS) ( Menkes et al., 1966 ; Horrocks, 1973 ; Yu and Yen, 1975 ; Chrast et al., 2011 ). The lipid content in optic (CNS) and sciatic (peripheral nervous system [PNS]) nerves is similar to other tissues of the nervous system, in that they contain the major gangliosides, phospholipids, cholesterol, cerebrosides, and sulfatides ( Peress and Boyle, 1975 ; Larrouquere-Regnier et al., 1979 ; Ganser et al., 1988a , 1988b ; McNally et al., 2007 ; Acar et al., 2012 ). GM1 ganglioside is enriched in myelin and has been used as an indicator of myelin content in brain tissue ( MacBrinn and O’Brien, 1969 ; Seyfried and Yu, 1980 ; Seyfried et al., 1984 ; Seyfried and Yu, 1984 ; Muse et al., 2001 ; Suzuki et al., 2001 ).…”