Literature DB >> 1651728

Quantitation of Mr 46000 and Mr 300000 mannose 6-phosphate receptors in human cells and tissues.

J Wenk1, A Hille, K von Figura.   

Abstract

A highly specific enzyme-linked immunosorbent assay for quantitation of the Mr 46000 Da and Mr 300000 Da mannose 6-phosphate receptors was developed. The assay allows to detect ng amounts of human mannose 6-phosphate receptors. Analysis of human cells and tissues revealed significant differences in their contents of the two mannose 6-phosphate receptors, normalized for total cell protein. The ratio of the two mannose 6-phosphate receptors also differed among cells and tissues, suggesting that their steady state concentrations are regulated independently.

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Year:  1991        PMID: 1651728

Source DB:  PubMed          Journal:  Biochem Int        ISSN: 0158-5231


  28 in total

1.  Overexpression of a rat kinase-deficient phosphoinositide 3-kinase, Vps34p, inhibits cathepsin D maturation.

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2.  Role of LAMP-2 in lysosome biogenesis and autophagy.

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Journal:  Mol Biol Cell       Date:  2002-09       Impact factor: 4.138

Review 3.  Gene therapy for the neurological manifestations in lysosomal storage disorders.

Authors:  Seng H Cheng
Journal:  J Lipid Res       Date:  2014-03-29       Impact factor: 5.922

4.  Liver production of sulfamidase reverses peripheral and ameliorates CNS pathology in mucopolysaccharidosis IIIA mice.

Authors:  Albert Ruzo; Miquel Garcia; Albert Ribera; Pilar Villacampa; Virginia Haurigot; Sara Marcó; Eduard Ayuso; Xavier M Anguela; Carles Roca; Judith Agudo; David Ramos; Jesús Ruberte; Fatima Bosch
Journal:  Mol Ther       Date:  2011-10-18       Impact factor: 11.454

5.  Clinical and metabolic correction of pompe disease by enzyme therapy in acid maltase-deficient quail.

Authors:  T Kikuchi; H W Yang; M Pennybacker; N Ichihara; M Mizutani; J L Van Hove; Y T Chen
Journal:  J Clin Invest       Date:  1998-02-15       Impact factor: 14.808

Review 6.  Pompe Disease: From Basic Science to Therapy.

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Journal:  Neurotherapeutics       Date:  2018-10       Impact factor: 7.620

7.  Alglucosidase alfa: Long term use in the treatment of patients with Pompe disease.

Authors:  Michael Beck
Journal:  Ther Clin Risk Manag       Date:  2009-09-24       Impact factor: 2.423

8.  A review of treatment of Pompe disease in infants.

Authors:  Yin-Hsiu Chien; Wuh-Liang Hwu
Journal:  Biologics       Date:  2007-09

9.  The pharmacological chaperone N-butyldeoxynojirimycin enhances enzyme replacement therapy in Pompe disease fibroblasts.

Authors:  Caterina Porto; Monica Cardone; Federica Fontana; Barbara Rossi; Maria Rosaria Tuzzi; Antonietta Tarallo; Maria Vittoria Barone; Generoso Andria; Giancarlo Parenti
Journal:  Mol Ther       Date:  2009-03-17       Impact factor: 11.454

10.  Replacement of alpha-galactosidase A in Fabry disease: effect on fibroblast cultures compared with biopsied tissues of treated patients.

Authors:  Jana Keslová-Veselíková; Helena Hůlková; Robert Dobrovolný; Befekadu Asfaw; Helena Poupetová; Linda Berná; Jakub Sikora; Lubor Golán; Jana Ledvinová; Milan Elleder
Journal:  Virchows Arch       Date:  2008-03-20       Impact factor: 4.064

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