Normal dolichol concentration in urine sediments from four patients with neuronal ceroid lipofuscinosis (Batten's disease)

Paton, Barbara C.; Poulos, A.

doi:10.1007/bf01799485

Cited by 6 publications

(2 citation statements)

References 11 publications

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“…They hypothesized that the aberrant metabolism of dolichol in the lysosome might be related to the neuronal dysfunction in these neurodegenerative disorders. On the other hand, Bennett and Paton failed to demonstrate the increased excretion of dolichol in the urinary sediments of the patients with NCL (Bennett et at, 1985;Paton and Poulos, 1987). The latter observation has raised a question on the specificity of dolichol accumulation in these disorders.…”

Section: Discussionmentioning

confidence: 87%

Elevated levels of dolichol in the brains of mucopolysaccharidosis and related disorders

Sakakihara

Imabayashi

Suzuki

et al. 1994

Molecular and Chemical Neuropathology

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The contents of total dolichol were measured in the cerebral cortex of various patients with lysosomal storage disorders, including mucopolysaccharidosis. Strikingly high levels of dolichol were demonstrated in GM1-gangliosides, Sanfilippo B syndrome, and a severe type of Hunter syndrome as well as neuronal ceroid-lipofuscinosis. An increased level of dolichol in cerebral cortex in neuronal ceroid-lipofuscinosis (NCL) was once regarded as pathognomonic for NCL. Our data, however, suggest that an increased level of dolichol in cerebral cortex is a nonspecific phenomenon related to some lysosomal dysfunction secondary to various neurodegenerative disorders.

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Section: Discussionmentioning

confidence: 87%

Elevated levels of dolichol in the brains of mucopolysaccharidosis and related disorders

Sakakihara

Imabayashi

Suzuki

et al. 1994

Molecular and Chemical Neuropathology

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“…The serum levels of fluidity, stability, permeability, and the fusion process dolichols were expressed as the sum of the three homologues of (Lai and Schutzbach 1984;Valtersson et al 1985). 18, 19, and 20 isoprene units. The sample preparation for the total dolichols included liberalevels of brain in patients suffering tion of dolichols from their fatty acid esters or protein complexes from Alzheimer's disease have been reported by Wolfe et al (1982), as well as Wolfe et al (1983), found raised urinary dolichols in NCLs, but this was not confirmed in two other studies (Bennett et al 1985;Paton and Poulos 1987). The increased urinary excretion of dolichols has also been discovered in another storage disease, the HermanskyPudlak syndrome .…”

Section: Introductionmentioning

confidence: 80%

Blood dolichol in lysosomal diseases

Jokelainen¹,

Salmela²,

Humaloja³

et al. 1992

Biochem. Cell Biol.

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Highly elevated serum total dolichol (free dolichol + dolichyl ester) concentrations have recently been found in two lysosomal storage diseases, aspartylglucosaminuria (AGU) and mannosidosis. The present study demonstrates that the increase of serum dolichol in AGU patients is caused by an increase of serum free dolichol. In 15 patients the mean serum level of free dolichol (227 +/- 16 ng/mL) was 1.9 times higher (p < 0.001) than that in healthy controls (120 +/- 6 ng/mL), while the amounts of dolichol fatty acid esters were similar in the patients and controls (110 +/- 9 vs. 118 +/- 6 ng/mL). In contrast, 10 patients with neuronal ceroid-lipofuscinosis (NCL) (three with infantile, four with juvenile, and three with variant late infantile NCL) had significantly (p < 0.01) lower mean serum levels of both free (79 +/- 5 ng/mL) and total (159 +/- 6 ng/mL) dolichol than age-adjusted healthy controls (free, 100 +/- 6 ng/mL; total, 206 +/- 14 ng/mL). Decreased blood dolichol has not been reported earlier for any other disease. We conclude that the increased serum free dolichol in AGU reflects disturbed lysosomal function and that the decreased free and esterified dolichols in NCLs speak against their presumed primary lysosomal nature.

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Dolichol and dolichyl phosphate in the neuronal ceroid-lipofuscinoses and other diseases

et al. 1988

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S. G.); Departments of Pathology (M. H.) and Neurology (J. P.) University of Helsinki, Helsinki, FinlandAlthough, compared to age-matched control samples, nonphosphorylated dolichols are significantly increased in the cerebral cortex of children with the late infantile and juvenile types of neurona1 ceroid-lipofuscinosis (NCL), doiichyl phosphates are increased to a much greater extent in infantile, late infantile, and juvenile forms of this disease group. Doiichyi phosphates in the cerebral cortex, expressed as a percentage of the combined nonphosphorylated and phosphorylated compounds, ranged from 59 to 85 (mean 71) in NCL, whereas in the non-NCL disease group the range is 18-36 (mean 25). This marked proportional increase in dolichyl phosphates is not unique to NCL but is also found in the brain of GMI,-gangliosidosis and Tay-Sachs disease patients. In the liver from NCL patients, dolichyl phosphates are not a major proportion of the total dolichol compounds (1-9%). However, in the kidney and heart, dolichyl phosphates are again markedly increased, and this is associated with large storage of ceroid. Although to a lesser extent than in NCL, dolichols and dolichyl phosphates are significantly increased over levels in age-matched control samples in the temporal cortex in Alzheimer disease. Our interpretation of these results is that storage in secondary in secondary lysosomes in NCL and the gangliosidoses leads to a decrease in the catabolism of dolichyl phosphate compounds in the Golgi saccules or primary l ysosomes.

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Normal dolichol concentration in urine sediments from four patients with neuronal ceroid lipofuscinosis (Batten's disease)

Cited by 6 publications

References 11 publications

Elevated levels of dolichol in the brains of mucopolysaccharidosis and related disorders

Elevated levels of dolichol in the brains of mucopolysaccharidosis and related disorders

Blood dolichol in lysosomal diseases

Dolichol and dolichyl phosphate in the neuronal ceroid-lipofuscinoses and other diseases

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