Abstract：
:Human skin fibroblasts deficient in peroxisome biogenesis were transformed by transfecting SV40 ori- DNA with the use of an electroporator, and the biochemical, immunocytochemical, and cytogenetic properties of the transformants were analyzed. Cells (1 x 10(6)) from a patient with Zellweger syndrome and one with neonatal adrenoleukodystrophy were suspended with 2 micrograms of SV40 ori- DNA in PBS; then a high-voltage pulse (2000 V, 30 microseconds) was generated two times. Several colonies expressing large T-antigen were picked up 4 weeks after transfection. Doubling time of the transformants was about half of that and the saturation density was 5 to 10 times greater than that of the parental cells. Biochemical abnormalities including defective lignoceric acid oxidation, dihydroxyacetone phosphate acyltransferase deficiency, and disturbed biosynthesis of peroxisomal beta-oxidation enzymes were preserved in the transformants. Peroxisomes were defective in all colonies, as determined by immunofluorescence staining using anti-catalase IgG. Cell fusion studies confirmed that the transformants belong to the same complementation groups as those of the parental cells. These transformed mutant cell lines are expected to be useful tools for investigating the pathogenesis of inherited diseases related to defects in peroxisome biogenesis.
journal_name
Exp Cell Resjournal_title
Experimental cell researchauthors
Okamoto H,Suzuki Y,Shimozawa N,Yajima S,Masuno M,Orii Tdoi
10.1016/0014-4827(92)90278-gkeywords:
subject
Has Abstractpub_date
1992-08-01 00:00:00pages
307-12issue
2eissn
0014-4827issn
1090-2422journal_volume
201pub_type
杂志文章abstract：:Hemidesmosomes are cell-to-matrix adhesion complexes anchoring keratinocytes to basement membranes. For the first time, we present a method to prepare a fraction from human cultured cells that are highly enriched in hemidesmosomal proteins. Using DJM-1 cells derived from human squamous cell carcinoma, accumulation of ...
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更新日期：1985-04-01 00:00:00
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更新日期：1998-07-10 00:00:00
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journal_title：Experimental cell research
pub_type： 杂志文章
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更新日期：1994-09-01 00:00:00
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doi：10.1006/excr.1999.4566
更新日期：1999-08-25 00:00:00
