Gangliosides in subacute sclerosing leukoencephalitis: isolation and fatty acid composition of nine fractions

Ledeen, Robert W.; Salsman, Kenneth; Cabrera, Mirel

doi:10.1016/s0022-2275(20)43153-0

Cited by 58 publications

(2 citation statements)

References 26 publications

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“…The latter were identified by GLC comparison with standards previously synthesized (Yu and Ledeen, 1969). This showed the absence of structures containing two sialic acids linked together by a (2-8) bond, such as are found in some of the brain gangliosides (Kuhn and Wiegandt, 1964;Klenk et al, 1967;Ledeen et al, 1968b). One brain disialoganglioside known to contain a NANAc(2-8)NANAc linkage (G2)3 was subjected to the same treatment and gave GLC peaks corresponding to both NANAc and NANAc-7.…”

Section: Resultsmentioning

confidence: 98%

Structures of gangliosides from bovine adrenal medulla

Price¹,

Kundu²,

Ledeen³

1975

Biochemistry

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Five gangliosides, accounting for over 95% of the total ganglioside fraction, were isolated from bovine adrenal medulla by preparative thin-layer chromatography and the carbohydrate structures determined by a combination of periodate oxidation and permethylation techniques. Partially methylated alditol acetates were generated from the neutral sugars of the fully methylated glycolipids and identified by gas-liquid chromatography. Substitution on N-acetylgalactosamine was determined by methanolysis of the permethylated ganglioside, acetylation of the products, and identification of the resulting substituted methyl glycosides by GLC. Periodate oxidation followed by borohydride reduction confirmed some of the linkages and demonstrated the absence of (2-8) linkages between sialic acid units. Mass spectrometry of the permethylated gangliosides gave information on sugar sequence at the nonreducing end.

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Section: Resultsmentioning

confidence: 98%

Structures of gangliosides from bovine adrenal medulla

Price¹,

Kundu²,

Ledeen³

1975

Biochemistry

Self Cite

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“…Ledeen et al subsequently confirmed that this structure was identical to that of the ganglioside G3A, which levels were high in the brain of a patient with subacute sclerosing leukoencephalitis. 21 The chemical formula of GD3 is C 72 H 129 N 3 O 29 and according to Svennerholm’s nomenclature, it corresponds to alpha- n -Acetylneuraminyl-2,8-alpha- n -acetylneuraminyl-2,3-beta- d -galactosyl-1,4-beta-glucosyl-1,1’-ceramide. 16 Some gangliosides, including GD3, can promote tumor-associated angiogenesis and strongly regulate cell adhesion, triggering tumor metastasis.…”

Section: Reviewmentioning

confidence: 99%

GD3 ganglioside is a promising therapeutic target for glioma patients.

Hein,

Baeza-Kallee,

Bertucci

et al. 2024

Neuro-Oncology Advances

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Glioblastoma is the most frequent and aggressive primary brain tumor in adults. Currently, no curative treatment is available. Despite first line treatment composed by the association of surgery, radiotherapy and chemotherapy, relapse remains inevitable in a median delay of 6 to 10 months. Improving patient management and developing new therapeutic strategies are therefore a critical medical need in neuro-oncology. Gangliosides are sialic acid-containing glycosphingolipids, the most abundant in the nervous system, representing attractive therapeutic targets. The ganglioside GD3 is highly expressed in neuroectoderm-derived tumors such as melanoma and neuroblastoma, but also in gliomas. Moreover, interesting results, including our own, have reported the involvement of GD3 in the stemness of glioblastoma cells. In this review, we will first describe the characteristics of the ganglioside GD3 and its enzyme, the GD3 synthase (GD3S), including their biosynthesis and metabolism. Then, we will detail their expression and role in gliomas. Finally, we will summarize the current knowledge regarding the therapeutic development opportunities against GD3 and GD3S.

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Trichopoliodystrophy

French¹,

Sherard²,

Lubell³

et al. 1972

Arch Neurol

131

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A new case of a rare disease previously named "a sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration" is reported. The name trichopoliodystrophy is suggested. Manifestations include seizures; developmental regression; light, sparse, coarse, stiff hair with monilethrix, trichorrhexis nodosa, and pili torti; deficient head growth; micrognathia; and hypotonia, neurophysiologically not localized to lower motor neurons or peripheral nerves. Tocopheral and ubiquinone were decreased in body fluids. White matter myelin and gray matter ganglioside were diminished. Whie matter ganglioside was increased without altered distribution. The myelin lipid pattern was immature. White matter phosphatidyl ethanolamine was deficit in longer chain length unsaturated fatty acids. Muscle glycogen was increased. Brain, muscle, and liver mitochondrial cytochrome a + a3 was diminished.Deficient terminal respiration may explain some of the findings. (26:229-244, 1972)

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Gangliosides in subacute sclerosing leukoencephalitis: isolation and fatty acid composition of nine fractions

Cited by 58 publications

References 26 publications

Structures of gangliosides from bovine adrenal medulla

Structures of gangliosides from bovine adrenal medulla

GD3 ganglioside is a promising therapeutic target for glioma patients.

Trichopoliodystrophy

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