Globoid

Diagnosis of Globoid Cell Leukodystrophy (Krabbe s Disease)". Blochem. Blophys. Res. Commun., (1971), 1363-1366. 

Ellis, W. G. Schneider E. L. McCulloch, J. R. Suzuki R. and Epstein, C. J. "Fetal Globoid Cell Leukodystrophy (Rrabbe s Disease) Pathological and Biochemical Examination". Arch. Neurol., (1973), 29, 253-257. 

Krabbe s leukodystrophy AR Galactocerebroside- P-galactosidase Globoid cells contain galactocerebroside see text 1,2, Ch. 40... 

Globoid cell leukodystrophy (Krabbe s disease) Galactosylceramidase Galactosylsphingosine Galactosylceramide... 

Globoid leukodystrophy Metachromatic leukodystrophy X-linked adrenoleukodystrophy Refsum s disease Cystinosis... 

Krivit, W., Peters, C. and Shapiro, E. G. Bone marrow transplantation as effective treatment of central nervous system disease in globoid cell leukodystrophy, metachro-matic leukodystrophy, adrenoleukodystrophy, mannosidosis, fucosidosis, aspartylglucosaminuria, Hurler, Maroteaux-Lamy, and Sly syndromes, and Gaucher disease type III. Curr. Opin. Neurol. 12 167-176,1999. 

These organelles occur in the endosperm of cereal grains and their structures are tissue specific. They are about 2-5 im in diameter and often contain globoid and occasionally crystalloid inclusions. Prolamin accumulates in small or large spherical bodies. Crystalline protein bodies are the sites of accumulation of nonprolamin storage proteins. 

Egerton-Warburton LM, West M, Lott JNA. Conservative allocation of globoid-held mineral nutrients inBanksia grandis (Proteaceae) seeds. CanJBot 1997 75 1951-1956. 

Figure 3. Transmission electron micrographs of mesophyll cells of dormant cotyledons of A, Cucurbita foetidissima B, Cucurbita pepo C, Cucurbita palmata D, Cucurbita digitata E, Apodanthera undulata" Note cell wall (W), protein body (P), spherosome (S), globoid (G), and crystalloid (X). In each micrograph, the bar represents five microns. Reproduced from reference 14.

Globoid bodies (glycolipid-leden marophages) in white matter of brain... 

A type with ovoid fruits was introduced into South-east Asia initially from India, a globoid-fruited type later from China and the typical European cultivars much later. However, it is cultivated in the region only as a domestic plant. It was also believed that an extract of the whole plant, or fruit, could preserve a person s spirit or essence after death and, for this reason, such compounds... 

Figure 5. Components and processes of a membrane-bound organelle, representing either a Protein Storage Vacuole (PSV) or a membrane-bound globoid found within a compound PSV V-PPase = vacuolar inorganic pyrophosphatase. V-ATPase = vacuolar ATPase. TIP = Tonoplast Intrinsic Protein. Ptdlns = phosphatidylinositol. DAG = diacylglycerol. Questionmarks indicate purely speculative aspects of the diagram.

Tominaga K, Matsuda J, Kido M, Naito E, Yokota I, Toida K, Ishimura K, Suzuki K, Kuroda Y (2004) Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the twitcher mouse model of globoid ceU leukodystrophy. J Neurosci Res 77 507-516... 

A significant proportion of patients with an LSD have neurological involvement, which can be manifested as developmental delay and behavioral changes (Table 2). The presence of specific findings may suggest the diagnosis (e.g., leucodystrophy in patients with metachromatic leucodystrophy or globoid cell leucodystrophy cherry... 

Intralysosomal substrate storage represents the initial insult to cells by-products of intermediary metabolism (e.g., psychosine in globoid cell leukodytrorphy), a disruption of normal lysosome function, and/or the consequent deficiency in recycling of certain substrates are putative disease events (Ballabio and Gieselmann,... 

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