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Literature DB >> 7107582

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

W A Gahl, F Tietze, N Bashan, R Steinherz, J D Schulman.

Abstract

Entities: Chemical

Mesh：

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Year: 1982 PMID： 7107582

Source DB: PubMed Journal: J Biol Chem ISSN： 0021-9258 Impact factor: 5.157

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47 in total

1. Identification and characterization of a lysosomal transporter for small neutral amino acids.

Authors: C Sagné; C Agulhon; P Ravassard; M Darmon; M Hamon; S El Mestikawy; B Gasnier; B Giros
Journal: Proc Natl Acad Sci U S A Date: 2001-06-05 Impact factor: 11.205

2. Lack of complementation in somatic cell hybrids between fibroblasts from patients with different forms of cystinosis.

Authors: O L Pellett; M L Smith; A A Greene; J A Schneider
Journal: Proc Natl Acad Sci U S A Date: 1988-05 Impact factor: 11.205

3. Cystine transport in purified rat liver lysosomes.

Authors: A J Jonas
Journal: Biochem J Date: 1986-06-15 Impact factor: 3.857

4. Role of adenosine triphosphate (ATP) and NaK ATPase in the inhibition of proximal tubule transport with intracellular cystine loading.

Authors: C Coor; R F Salmon; R Quigley; D Marver; M Baum
Journal: J Clin Invest Date: 1991-03 Impact factor: 14.808

5. N-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis.

Authors: Luciana Pache de Faria Guimaraes; Antonio Carlos Seguro; Maria Heloisa Mazzola Shimizu; Letícia Aparecida Lopes Neri; Nairo Massakasu Sumita; Ana Carolina de Bragança; Rildo Aparecido Volpini; Talita Rojas Cunha Sanches; Fernanda Andrade Macaferri da Fonseca; Carlos Alberto Moreira Filho; Maria Helena Vaisbich
Journal: Pediatr Nephrol Date: 2013-12-11 Impact factor: 3.714

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

1. Identification and characterization of a lysosomal transporter for small neutral amino acids.

2. Lack of complementation in somatic cell hybrids between fibroblasts from patients with different forms of cystinosis.

3. Cystine transport in purified rat liver lysosomes.

4. Role of adenosine triphosphate (ATP) and NaK ATPase in the inhibition of proximal tubule transport with intracellular cystine loading.

5. N-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis.

6. Metabolism of pantethine in cystinosis.

7. Transport of glutathione, as gamma-glutamylcysteinylglycyl ester, into liver and kidney.

8. Inhibitors of protein synthesis also inhibit lysosomal proteolysis. Studies using cystinotic fibroblasts.

9. CTNS mutations in an American-based population of cystinosis patients.

10. An improved method for heterozygote detection of cystinosis, using polymorphonuclear leukocytes.