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The uptake of cystine by vesicles prepared from rat kidney brush borders occurs by two distinct transport systems. The higher affinity system is inhibited by the dibasic amino acids lysine, arginine, and ornithine. The lower affinity system, unaffected by dibasic amino acids, appears to correspond to that observed by studying uptake of cystine by kidney(More)
Uptake of L-cystine by brush-border membrane vesicles isolated from rat renal-cortical tissue was time-dependent and occurred in the absence of cystine reduction. A significant capacity for vesicular binding of cystine was observed. The amount bound increased with time of incubation and could be displaced by thiol reagents. At early time points, cystine(More)
The effect of cystine dimethylester on the renal handling of phosphate, glucose, alpha-amino nitrogen, amino acids, and protein in vivo and on the uptake of lysine, glycine, taurine, and alpha-methyl glucoside by isolated renal tubules in vitro was studied in adult male rats. Parenteral administration of 400 mumol twice a day for four days of cystine(More)
OBJECTIVE The study examined whether the prescription practices of clinicians in psychiatric emergency services differed for African-American patients. Prescription of antipsychotic medications and its relation to quality of care was a particular focus. METHODS Data from 442 independently observed evaluations of patients in psychiatric emergency services(More)
We report a controlled study of intellectual outcome in 16 children with maple syrup urine disease (MSUD) that compares the outcome of MSUD diagnosed after symptoms became apparent with that of MSUD diagnosed prospectively and in unaffected siblings and parents. The mean IQ (+/- SD) score in the children with classic MSUD was 78 +/- 24; however, there were(More)
While dietary galactose restriction eliminates the life-threatening complications of classic galactosemia, central nervous system and ovarian disease are still evident in these patients, despite milk restriction. Because of the possibility that reduced tissue levels of uridine diphosphate galactose (UDPgalactose), the product of the deficient enzyme,(More)
A ten-year follow-up study of 393 seriously mentally ill sheltered-care residents examined the type and degree of handicap characterizing the sample and estimated effects of ten-year residence. Results showed higher levels of helper-supported social functioning and of physical and mental health, accompanied by significant reductions in independent social(More)
A patient is described in whom lactic acidosis of very severe degree was found to coincide with the presence of beta-methylcrotonic acid and rho-hydroxyphenyllactic acid in urine in large amounts, while beta-hydroxyisovaleric acid was found to be a relatively minor excretion product. Beta-methylcrotonic acid is demonstrated, for the first time, to be(More)
We have developed a sensitive method that employs high-performance liquid chromatography to separate and quantitate uridine diphosphogalactose (UDPGal) and uridine diphosphoglucose (UDPGlu) in human red blood cells. The trichloracetic acid extracts of red blood cells were chromatographed using a Dionex CarboPac anion-exchange resin and a 20-40% potassium(More)