Literature DB >> 3165268

Synthesis and stability of steroid sulfatase in fibroblasts from multiple sulfatase deficiency.

J T Conary1, A Hasilik, K von Figura.   

Abstract

Multiple sulfatase deficiency is a lysosomal storage disorder, which can be divided into group I with severe and group II with moderate deficiencies in sulfatases. Antibodies raised against steroid sulfatase purified from human placenta were used to follow the biosynthesis and stability of this enzyme in multiple sulfatase-deficiency fibroblasts. Fibroblasts from both groups synthesized steroid sulfatase of apparently normal size and stability, while the apparent rate of enzyme synthesis and catalytic properties of steroid sulfatase were affected to a variable extent. Cell lines were observed, that synthesized normal amounts of steroid-sulfatase polypeptides, which were catalytically inactive, as well as cell lines that synthesized diminished amounts of catalytically active steroid sulfatase.

Entities:  

Mesh:

Substances:

Year:  1988        PMID: 3165268     DOI: 10.1515/bchm3.1988.369.1.297

Source DB:  PubMed          Journal:  Biol Chem Hoppe Seyler        ISSN: 0177-3593


  6 in total

1.  SUMF1 mutations affecting stability and activity of formylglycine generating enzyme predict clinical outcome in multiple sulfatase deficiency.

Authors:  Lars Schlotawa; Eva Charlotte Ennemann; Karthikeyan Radhakrishnan; Bernhard Schmidt; Anupam Chakrapani; Hans-Jürgen Christen; Hugo Moser; Beat Steinmann; Thomas Dierks; Jutta Gärtner
Journal:  Eur J Hum Genet       Date:  2011-01-12       Impact factor: 4.246

2.  Human N-acetylgalactosamine-4-sulphatase biosynthesis and maturation in normal, Maroteaux-Lamy and multiple-sulphatase-deficient fibroblasts.

Authors:  J A Taylor; G J Gibson; D A Brooks; J J Hopwood
Journal:  Biochem J       Date:  1990-06-01       Impact factor: 3.857

3.  alpha-L-iduronidase in normal and mucopolysaccharidosis-type-I human skin fibroblasts.

Authors:  J A Taylor; G J Gibson; D A Brooks; J J Hopwood
Journal:  Biochem J       Date:  1991-02-15       Impact factor: 3.857

4.  Correction of human mucopolysaccharidosis type-VI fibroblasts with recombinant N-acetylgalactosamine-4-sulphatase.

Authors:  D S Anson; J A Taylor; J Bielicki; G S Harper; C Peters; G J Gibson; J J Hopwood
Journal:  Biochem J       Date:  1992-06-15       Impact factor: 3.857

5.  Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs.

Authors:  W Rommerskirch; K von Figura
Journal:  Proc Natl Acad Sci U S A       Date:  1992-04-01       Impact factor: 11.205

6.  Two site-directed mutations abrogate enzyme activity but have different effects on the conformation and cellular content of the N-acetylgalactosamine 4-sulphatase protein.

Authors:  D A Brooks; D A Robertson; C Bindloss; T Litjens; D S Anson; C Peters; C P Morris; J J Hopwood
Journal:  Biochem J       Date:  1995-04-15       Impact factor: 3.857

  6 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.