Literature DB >> 4269326

Sanfilippo disease type B: enzyme replacement and metabolic correction in cultured fibroblasts.

J S O'Brien, A L Miller, A W Loverde, M L Veath.   

Abstract

alpha-Acetylglucosaminidase, purified from human placent, corrected the defect in mucopolysaccharide degradation when added to culture fibroblasts from patients with Sanfilippo disease type B. A small cellular concentration of enzyme gave a large corrective effect. The half-life of disappearance of enzyme activity was 4 to 7 days.

Entities:  

Mesh：

Substances：

Year:  1973        PMID： 4269326     DOI： 10.1126/science.181.4101.753

Source DB:  PubMed          Journal:  Science        ISSN： 0036-8075            Impact factor:   47.728


  14 in total

Review 1.  Replacement therapy in the mucopolysaccharidoses.

Authors:  M F Dean
Journal:  J Clin Pathol Suppl (R Coll Pathol)       Date:  1978

Review 2.  New biotechnological and nanomedicine strategies for treatment of lysosomal storage disorders.

Authors:  Silvia Muro
Journal:  Wiley Interdiscip Rev Nanomed Nanobiotechnol       Date:  2010 Mar-Apr

3.  Excretion-reuptake route of beta-hexosaminidase in normal and I-cell disease cultured fibroblasts.

Authors:  G D Vladutiu; M C Rattazzi
Journal:  J Clin Invest       Date:  1979-04       Impact factor: 14.808

4.  The use of quantiatative cytochemical analyses in rapid prenatal detection and somatic cell genetic studies of metabolic diseases.

Authors:  H Galjaard; A Hoogeveen; W Keijzer; E De Wit-Verbeek; C Vlek-Noot
Journal:  Histochem J       Date:  1974-09

Review 5.  Neural stem cell transplantation as a therapeutic approach for treating lysosomal storage diseases.

Authors:  Lamya S Shihabuddin; Seng H Cheng
Journal:  Neurotherapeutics       Date:  2011-10       Impact factor: 7.620

6.  Enzyme replacement therapy by fibroblast transplantation: long-term biochemical study in three cases of Hunter's syndrome.

Authors:  M F Dean; R L Stevens; H Muir; P F Benson; L R Button; R L Anderson; A Boylston; J Mowbray
Journal:  J Clin Invest       Date:  1979-01       Impact factor: 14.808

Review 7.  Enzyme replacement and enhancement therapies for lysosomal diseases.

Authors:  R J Desnick
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

8.  Human beta-glucuronidase deficiency mucopolysaccharidosis: identification of cross-reactive antigen in cultured fibroblasts of deficient patients by enzyme immunoassay.

Authors:  C E Bell; W S Sly; F E Brot
Journal:  J Clin Invest       Date:  1977-01       Impact factor: 14.808

9.  Selective noncompetitive assimilation of bovine testicular beta-galactosidase and bovine liver beta-glucuronidase by generalized gangliosidosis fibroblasts.

Authors:  V Hieber; J Distler; R Myerowitz; R D Schmickel; G W Jourdian
Journal:  J Clin Invest       Date:  1980-04       Impact factor: 14.808

10.  Caprine beta-D-mannosidosis: characterization of a model lysosomal storage disorder.

Authors:  R D Pearce; J W Callahan; P B Little; L R Klunder; J T Clarke
Journal:  Can J Vet Res       Date:  1990-01       Impact factor: 1.310
View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.