A digital computer simulation of copper metabolism was used to simulate human copper metabolism. The simulation agrees well with the normal data extant. Wilson's disease (hepatolenticular degeneration) and Menkes' disease (steely-hair syndrome) were simulated. Simulation of the unavailability of accumulated liver copper simulated Wilson's disease if it was assumed that the increased urinary excretion was due to induction of an enzymic mechanism for enhanced excretion. This would be consistent with the genetic defect causing only the sequestering of unavailable copper in the liver. Other genetic defects need not be present. Menkes' disease is also a genetic disease affecting the newborn. It was simulated successfully as a defect in absorption of copper from the gastrointestinal tract.
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NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314
BERATIS, NG
PRICE, P
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NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314
PRICE, P
LABADIE, GU
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NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314
LABADIE, GU
HIRSCHHORN, K
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NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314NEW YORK STATE INST BAS RES MENTAL RETARDAT,DEPT HUMAN GENET,STATEN ISL,NY 10314