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Subcellular localisation and processing of non‐specific lipid transfer protein are not aberrant in Rhizomelic Chondrodysplasia Punctata fibroblasts
Author(s) -
Heikoop Judith C.,
Ossendorp Bernadette C.,
Wanders Ronald J.A.,
Wirtz Karel W.A.,
Tager Joseph M.
Publication year - 1992
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/0014-5793(92)80247-e
Subject(s) - peroxisome , thiolase , plant lipid transfer proteins , peroxisomal disorder , chondrodysplasia punctata , phospholipid transfer protein , microbiology and biotechnology , biology , cell fractionation , subcellular localization , biochemistry , anatomy , enzyme , cytoplasm , gene , phospholipid , membrane
The import into peroxisomes and maturation of peroxisomal 3‐oxoacyl‐CoA thiolase are impaired in patients with the Rhizomelic form of Chondrodysplasia Punctata (RCDP). Here we show by means of immunoblotting and subcellular fractionation that non‐specific lipid transfer protein (nsLTP), another peroxisomal protein synthesised as a larger precursor, is localised in peroxisomes and is present as the mature protein in RCDP fibroblasts. Thus the component of the import machinery defective in RCDP is not required for the import of nsLTP into peroxisomes.