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  • 1
    Electronic Resource
    Electronic Resource
    Absence of a role of gamma-glutamyl transpeptidase in the transport of amino acids by rat renal brushborder membrane vesicles (1984)
    Springer
    The journal of membrane biology 80 (1984), S. 167-173 
    Details
    ISSN: 1432-1424
    Keywords: AT-125 ; gamma-glutamyl transpeptidase ; renal brushborder membrane ; amino acids transport
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Summary The role of the enzyme, gamma-glutamyl transpeptidase on the uptake of amino acids by the brushborder membrane of the rat proximal tubule was examined by inhibiting it with AT-125 (l-[αS, 5S]-α-amino-3-chloro-4,5-dihydro-5-isoxazoleacetic acid). AT-125 inhibited 98% of the activity of gamma-glutamyl transpeptidase when incubated for 20 min at 37°C with rat brushborder membrane vesicles. AT-125 given to ratsin vivo inhibited 90% of the activity of gamma-glutamyl transpeptidase in subsequently isolated brushborder membrane vesicles from these animals. AT-125 inhibition of gamma-glutamyl transpeptidase bothin vivo andin vitro had no effect on the brushborder membrane uptake of cystine. Similarly, there was no effect of gamma-glutamyl transpeptidase inhibition by AT-125 on glutamine, proline, glycine, methionine, leucine or lysine uptake by brushborder membrane vesicles. Furthermore, the uptake of cystine by isolated rat renal cortical tubule fragments, in which the complete gamma-glutamyl cycle is present, was unaffected by AT-125 inhibition of gamma-glutamyl transpeptidase. Therefore, in the two model systems studied, gamma-glutamyl transpeptidase did not appear to play a role in the transport of amino acids by the renal brushborder membrane.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01868772
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  • 2
    Electronic Resource
    Electronic Resource
    Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination (2003)
    [s.l.] : Nature Publishing Group
    Nature genetics 34 (2003), S. 413-420 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, leads to chronic renal failure in children. The genes mutated in NPHP1 and NPHP4 have been identified, and a gene locus associated with infantile nephronophthisis (NPHP2) was mapped. The kidney phenotype of NPHP2 combines ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/ng1217
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  • 3
    Electronic Resource
    Electronic Resource
    The peritoneal equilibration test in children (1993)
    Springer
    Pediatric nephrology 7 (1993), S. 731-734 
    Details
    ISSN: 1432-198X
    Keywords: Chronic renal failure ; Peritoneal dialysis ; Continuous ambulatory peritoneal dialysis ; Continuous cyclic peritoneal dialysis ; Peritoneal equilibration test
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Eight children, aged 15 months to 17 years 9 months, maintained by continuous ambulatory peritoneal dialysis (CAPD)/continuous cycling peritoneal dialysis and nine adults, aged 20–59 years, managed by CAPD were compared using a standardized peritoneal dialysis protocol, the peritoneal equilibration test (PET). The peritoncal glucose concentration tended to equilibrate with the serum glucose more rapidly in children, but the percentage of the glucose load absorbed was not different between the two age groups. There was an inverse trend between the percentage of glucose absorbed and age in children. Peritoneal creatinine clearance scaled to surface area in children was significantly less than that of the adults; however, the clearances became similar when adjusted for body weight. Peritoneal creatinine clearace scaled to surface area bore a positive and significant relationship to age which, when expressed per kilogram body weight, disappeared. Children had a significantly higher dialysate/plasma (D.P) creatinine ratio after the first 2 h of the PET, but this ratio approached unity by 4 and was not different from adults. The fractional change in the creatinine D/P ratio during the PET was not different between the two age groups. Drain volume adjusted to surface area was significantly less in children than adults. This difference was reversed when drain volume was factored by weight. Similarly drain volume scaled to surface area demonstrated a significant and positive relationship to age, which disappeared when drain volume was expressed per kilogram body weight. Ultrafiltration, whether factored by weight or scaled to surface area, did not differ between the two groups. The post-PEt residual volume corrected for body weight was significantly larger in the children, but bore no relationship to age. It is possible that this larger residual volume in children functions as a tidal volume enhancing solute equilibration.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01213336
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  • 4
    Electronic Resource
    Electronic Resource
    Effect of cystine loading on substrate oxidation by rat renal tubules (1990)
    Springer
    Pediatric nephrology 4 (1990), S. 236-239 
    Details
    ISSN: 1432-198X
    Keywords: Cystine ; Substrate oxidation ; Tubular metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Isolated rat renal tubules were loaded with cystine by incubating them with 2 mM cystine dimethylester. The oxidation of 1 mM glucose and lactate was significantly decreased after 20 and 30 min of incubation, in the cystine-loaded tubules compared with control tubules. The oxidation of 1 mM butyrate was significantly decreased after 10 and 30 min of incubation in the cystine-loaded tubules. Cystine loading decreased the oxidation of 1 mM succinate at all time points examined. The O2 consumption of renal tubules was reduced 59% with cystine loading by the addition of 2 mM cystine dimethylester, and by 37% with 1 mM cystine dimethylester. These data indicate that loading normal renal tubule cells with cystine impairs their ability to oxidize metabolic fuels. This impairment in metabolism may explain the decreased transport observed previously in cystine-loaded tubules and may have implications for the human disorder, cystinosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00857662
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  • 5
    Electronic Resource
    Electronic Resource
    Effect of cystine loading and cystine dimethylester on renal brushborder membrane transport (1990)
    Springer
    Bioscience reports 10 (1990), S. 455-459 
    Details
    ISSN: 1573-4935
    Keywords: cystine ; cystine dimethylester ; brushborder membrane ; renal transport ; cystinosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Abstract The effect of loading renal tubule cells with cystine was studied by incubating them with cystine dimethylester. Proline uptake into brushborder membrane vesicles isolated from the cystine loaded cells was not different from that observed into brushborder vesicles isolated from tubules incubated in buffer alone. Incubating brushborder membranes with 2 mM cystine dimethylester for 10 minutes reduced the uptake of proline by 27% after 15 seconds of incubation and by 21% after 60 seconds of incubation. There was no effect after 20 minutes of incubation. Pre-incubating brushborder membrane vesicles with cystine dimethylester had no statistically significant effect on the affinity of priline for the carrier, but did reduce the maximal rate of proline uptake by 49%.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01152292
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  • 6
    Electronic Resource
    Electronic Resource
    Cystine-glutamate transport interactions in rat renal cortical tubules, brushborder vesicles, and cultured renal tubule cells (1986)
    Springer
    Bioscience reports 6 (1986), S. 113-119 
    Details
    ISSN: 1573-4935
    Keywords: amino acid transport ; renal tubule ; brushborder membrane ; cystine ; glutamate
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology
    Notes: Abstract Glutamate had no significant effect on the uptake of 0.025 mM cystine by isolated rat renal cortical tubules and brushborder membrane vesicles in contrast to lysine which significantly inhibits cystine transport. Glutamate, however, markedly inhibited cystine uptake by rat renal tubule cells grown in a serum-free, hormonally defined media for 5 days. Lysine also inhibited cystine transport in these cultured renal tubule cells.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01145186
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