Enveloped virus acquires membrane defect when passaged in fibroblasts from I-cell disease patients.
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چکیده
منابع مشابه
Defective catabolism of low-density lipoprotein by fibroblasts from patients with I-cell disease.
Skin fibroblast cultures from patients with I-cell disease (mucolipidosis II) are characterized by multiple lysosomal enzyme deficiencies The present studies deal with the consequences of these deficiencies with respect to the metabolism of plasma low-density lipoproteins. Degradation of the protein moiety was defective in I-cells compared with control cells, but the binding and internalization...
متن کاملHydrolases from I-Cell Cultured Fibroblasts
I-cell cultured fibroblasts secrete excessive amounts of N-acetyl-fl-D-hexosaminidase and a -L-fucosidase into the culture media as compared with normal fibroblasts. Addition of tunicamycin or cycloheximide at doses that inhibit the incorporation of [3Hlmannose (60-80%) and [14C]leucine (40-50%) into trichloroacetic acid-precipitable material decreased the secretion of these I-cell hydrolases t...
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Transformation by Rous sarcoma virus (RSV) has been reported to block the expression of differentiated cell products in chicken cells. The expression of these proteins may or may not be suppressed when temperature-sensitive mutants are shifted from the nonpermissive to the permissive temperature. A general characteristic of cellular transformation is the disruption of the microfilament system. ...
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Transformation by Rous sarcoma virus (RSV) has been reported to block the expression of differentiated cell products in chicken cells. The expression of these proteins may or may not be suppressed when temperature-sensitive mutants are shifted from the nonpermissive to the permissive temperature. A general characteristic of cellular transformation is the disruption of the microfilament system. ...
متن کاملI-Cell Disease with GNPTAB Gene Mutation
I-cell disease is a rare inherited metabolic disorder resulting from a defective phosphotransferase, characterized by coarse facial features, skeletal abnormalities and mental retardation. As clinical features of this condition mimic that of Hurler disease mutation studies help in the diagnosis. We present a case of I-cell disease in a neonate with GNPTAB gene mutation.
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 1976
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.73.7.2443