Subcellular localisation and processing of non‐specific lipid transfer protein are not aberrant in Rhizomelic Chondrodysplasia Punctata fibroblasts

FEBS Letters

Heikoop, Judith C.²  Tager, Joseph M.³  Ossendorp, Bernadette C.⁴  Wanders, Ronald J.A.¹  Wirtz, Karel W.A.⁴ 

[1] Department of Pediatrics, University Hospital, Academic Medical Centre, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands;EC Slater Institute for Biochemical Research, University of Amsterdam, Academic Medical Centre, Meibergdreef 15, 1105 AZ, Amsterdam, The Netherlands;Institute of Medical Biochemistry, University of Bari, Piazza Giulio Cesare, 70124 Bari, Italy;Centre for Biomembranes and Lipid Enzymology, State University of Utrecht, Transitorium III, Padualaan 8, 3584 CH Utrecht, The Netherlands

关键词: Peroxisome;    Rhizomelic Chondrodysplasia Punctata;    Non-specific lipid transfer protein;    Sterol carrier protein 2-;    Peroxisomal 3-oxoacyl-CoA thiolase;   

DOI  :  10.1016/0014-5793(92)80247-E

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown