Infantile Niemann-Pick Disease

Ganglioside Abnormalities in Multiple Sclerosis

“…Plaque gangliosides, unlike those of MS white matter, showed many departures from normal white matter in their TLC pattern. Decrease of G,, the major monosialoganglioside, was one notable feature and was consistent with previous descriptions of this substance as the major myelin ganglioside of human brain (KAMOSHITA et al, 1969;. The fact that it is not entirely absent from plaque tissue suggests it is also a component of axons and/or astrocytes.…”

Electron microscopy of lung in niemann‐pick disease

“…In Tay-Sachs disease the periodicity in similar bodies is 6 nm. In Niemann-Pick disease rather different bodies have been described in the liver by Volk and Wallace (1966) and in the cerebellum by Kamoshita et al (1969). Whether these bodies are comparable to those in the present study or in the cases of Lynn and Terry, and of Tanaka et al is debatable.…”

Identification of (N-acyl)ethanolamine phosphoglycerides and acylphosphatidylglycerol as the phospholipids which disappear as Dictyostelium discoideum cells aggregate

“…In rat liver (Wherrett & Huterer, 1972), rabbit alveolar macrophages (Mason et al, 1972), and BHK cells , the secondary lysosomes are enriched with bis(acylglycerol) phosphate. It is also associated with the secondary lysosomes filled with multilamellar structures in patients with type A Niemann Pick disease (Rouser et al, 1968;Kamoshita et al, 1969) and in livers of drug-treated rats . APG has been identified in Salmonella typhimurium (Olsen & Ballou, 1971), degenerating BHK cells (Somerharju et al, 1977), and in liver lysosomes of chloroquine-treated rats .…”