Literature DB >> 2702086

Has the basic metabolic defect of cystinosis been discovered?

J A Schneider1.   

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Year:  1989        PMID: 2702086     DOI: 10.1007/BF00859624

Source DB:  PubMed          Journal:  Pediatr Nephrol        ISSN: 0931-041X            Impact factor:   3.714


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  12 in total

1.  Cystinotic fibroblasts accumulate cystine from intracellular protein degradation.

Authors:  J G Thoene; R G Oshima; D G Ritchie; J A Schneider
Journal:  Proc Natl Acad Sci U S A       Date:  1977-10       Impact factor: 11.205

2.  Proton-translocating ATPase and lysosomal cystine transport.

Authors:  A J Jonas; M L Smith; W S Allison; P K Laikind; A A Greene; J A Schneider
Journal:  J Biol Chem       Date:  1983-10-10       Impact factor: 5.157

3.  Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes.

Authors:  W A Gahl; F Tietze; N Bashan; R Steinherz; J D Schulman
Journal:  J Biol Chem       Date:  1982-08-25       Impact factor: 5.157

4.  Characterization of carrier-mediated transport systems for small neutral amino acids in human fibroblast lysosomes.

Authors:  R L Pisoni; K S Flickinger; J G Thoene; H N Christensen
Journal:  J Biol Chem       Date:  1987-05-05       Impact factor: 5.157

5.  Characteristics of a lysosomal membrane transport system for tyrosine and other neutral amino acids in rat thyroid cells.

Authors:  J Bernar; F Tietze; L D Kohn; I Bernardini; G S Harper; E F Grollman; W A Gahl
Journal:  J Biol Chem       Date:  1986-12-25       Impact factor: 5.157

6.  ATP-dependent lysosomal cystine efflux is defective in cystinosis.

Authors:  A J Jonas; M L Smith; J A Schneider
Journal:  J Biol Chem       Date:  1982-11-25       Impact factor: 5.157

7.  Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis.

Authors:  W A Gahl; N Bashan; F Tietze; I Bernardini; J D Schulman
Journal:  Science       Date:  1982-09-24       Impact factor: 47.728

8.  Defect in vitamin B12 release from lysosomes: newly described inborn error of vitamin B12 metabolism.

Authors:  D S Rosenblatt; A Hosack; N V Matiaszuk; B A Cooper; R Laframboise
Journal:  Science       Date:  1985-06-14       Impact factor: 47.728

9.  Detection and characterization of carrier-mediated cationic amino acid transport in lysosomes of normal and cystinotic human fibroblasts. Role in therapeutic cystine removal?

Authors:  R L Pisoni; J G Thoene; H N Christensen
Journal:  J Biol Chem       Date:  1985-04-25       Impact factor: 5.157

10.  Studies of lysosomal sialic acid metabolism: retention of sialic acid by Salla disease lysosomes.

Authors:  A J Jonas
Journal:  Biochem Biophys Res Commun       Date:  1986-05-29       Impact factor: 3.575

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  1 in total

1.  Pathogenesis and Host Immune Response in Leprosy.

Authors:  Hadida Yasmin; Praveen Mathews Varghese; Sanjib Bhakta; Uday Kishore
Journal:  Adv Exp Med Biol       Date:  2021       Impact factor: 2.622

  1 in total

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