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Treatment of adrenoleukodystrophy with bone marrow transplantation.

One or more mevalonate derivatives of non-sterol type have been proposed to be of indispensable importance for cell growth. Conceivable mevalonate-dependent mechanisms involved in growth control are farnesylation of Ras proteins, regulation of c-myc expression, and N-linked glycosylation of the IGF-... Full description

Journal Title: Acta paediatrica (Oslo Norway : 1992), May 1997, Vol.86(5), pp.484-492
Main Author: Malm, G
Other Authors: Ringdén, O , Anvret, M , Von Döbeln, U , Hagenfeldt, L , Isberg, B , Knuutila, S , Nennesmo, I , Winiarski, J , Marcus, C
Format: Electronic Article Electronic Article
Language: English
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ID: ISSN: 0803-5253
Link: http://search.proquest.com/docview/79048919/?pq-origsite=primo
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title: Treatment of adrenoleukodystrophy with bone marrow transplantation.
format: Article
creator:
  • Malm, G
  • Ringdén, O
  • Anvret, M
  • Von Döbeln, U
  • Hagenfeldt, L
  • Isberg, B
  • Knuutila, S
  • Nennesmo, I
  • Winiarski, J
  • Marcus, C
subjects:
  • Bone Marrow Transplantation–Methods
  • Child–Diagnosis
  • Child, Preschool–Therapy
  • Dietary Fats–Therapy
  • Disease Progression–Therapy
  • Fatal Outcome–Therapy
  • Humans–Therapy
  • Magnetic Resonance Imaging–Therapy
  • Male–Therapy
  • Neuropsychological Tests–Therapy
  • Peroxisomal Disorders–Therapy
  • Dietary Fats
ispartof: Acta paediatrica (Oslo, Norway : 1992), May 1997, Vol.86(5), pp.484-492
description: One or more mevalonate derivatives of non-sterol type have been proposed to be of indispensable importance for cell growth. Conceivable mevalonate-dependent mechanisms involved in growth control are farnesylation of Ras proteins, regulation of c-myc expression, and N-linked glycosylation of the IGF-1 receptor. The latter mechanism might be rate-limited by dolichyl phosphate, which acts as a donor of oligosaccharides in glycoprotein synthesis in the endoplasmic reticulum. In order to study the significance for cell proliferation of the three aforementioned mevalonate-dependent processings, their inhibitory response due to mevalonate deprivation was explored and compared with the effect on DNA synthesis in the malignant melanoma cell line SK-MEL-2. We found that mevalonate depletion due to treatment with 3 microM lovastatin for 24 h, which efficiently growth-arrested the cells, hardly at all affected the expression of c-myc, and although Ras prenylation was inhibited by 50%, the most pronounced effect of lovastatin was seen on N-linked glycosylation of IGF-1 receptors, which was inhibited by more than 95%. The order and magnitude of the decreased IGF-1 receptor glycosylation, which was followed by a decreased expression of IGF-1 receptors at the cell membrane, correlated well with the inhibition of DNA synthesis. We investigated whether dolichol, and in particular dolichyl phosphate, through its participation in N-linked glycosylation, act as regulators of IGF-1 receptor expression. First, we could confirm that exogenous dolichol became phosphorylated and in this form took part in the glycosylation processing. Secondly, we showed that dolichyl phosphate, in a dose-dependent manner, could increase the number of IGF-1 receptors at the cell membrane, simultaneously as DNA synthesis was stimulated. Taken together, our results provide direct evidence for an important role of dolichyl phosphate as a regulator of cell growth through limiting N-linked glycosylation of the IGF-1 receptor.
language: eng
source:
identifier: ISSN: 0803-5253
fulltext: fulltext
issn:
  • 08035253
  • 0803-5253
url: Link


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titleTreatment of adrenoleukodystrophy with bone marrow transplantation.
creatorMalm, G ; Ringdén, O ; Anvret, M ; Von Döbeln, U ; Hagenfeldt, L ; Isberg, B ; Knuutila, S ; Nennesmo, I ; Winiarski, J ; Marcus, C
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identifierISSN: 0803-5253
subjectBone Marrow Transplantation–Methods ; Child–Diagnosis ; Child, Preschool–Therapy ; Dietary Fats–Therapy ; Disease Progression–Therapy ; Fatal Outcome–Therapy ; Humans–Therapy ; Magnetic Resonance Imaging–Therapy ; Male–Therapy ; Neuropsychological Tests–Therapy ; Peroxisomal Disorders–Therapy ; Dietary Fats
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descriptionOne or more mevalonate derivatives of non-sterol type have been proposed to be of indispensable importance for cell growth. Conceivable mevalonate-dependent mechanisms involved in growth control are farnesylation of Ras proteins, regulation of c-myc expression, and N-linked glycosylation of the IGF-1 receptor. The latter mechanism might be rate-limited by dolichyl phosphate, which acts as a donor of oligosaccharides in glycoprotein synthesis in the endoplasmic reticulum. In order to study the significance for cell proliferation of the three aforementioned mevalonate-dependent processings, their inhibitory response due to mevalonate deprivation was explored and compared with the effect on DNA synthesis in the malignant melanoma cell line SK-MEL-2. We found that mevalonate depletion due to treatment with 3 microM lovastatin for 24 h, which efficiently growth-arrested the cells, hardly at all affected the expression of c-myc, and although Ras prenylation was inhibited by 50%, the most pronounced effect of lovastatin was seen on N-linked glycosylation of IGF-1 receptors, which was inhibited by more than 95%. The order and magnitude of the decreased IGF-1 receptor glycosylation, which was followed by a decreased expression of IGF-1 receptors at the cell membrane, correlated well with the inhibition of DNA synthesis. We investigated whether dolichol, and in particular dolichyl phosphate, through its participation in N-linked glycosylation, act as regulators of IGF-1 receptor expression. First, we could confirm that exogenous dolichol became phosphorylated and in this form took part in the glycosylation processing. Secondly, we showed that dolichyl phosphate, in a dose-dependent manner, could increase the number of IGF-1 receptors at the cell membrane, simultaneously as DNA synthesis was stimulated. Taken together, our results provide direct evidence for an important role of dolichyl phosphate as a regulator of cell growth through limiting N-linked glycosylation of the IGF-1 receptor.
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titleTreatment of adrenoleukodystrophy with bone marrow transplantation.
authorMalm, G ; Ringdén, O ; Anvret, M ; Von Döbeln, U ; Hagenfeldt, L ; Isberg, B ; Knuutila, S ; Nennesmo, I ; Winiarski, J ; Marcus, C
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