ABNORMAL ACCUMULATION OF PORPHYRIN DERIVATIVES IN THE KIDNEYS OF LONG-EVANS CINNAMON RATS, AS EVIDENCED BY MICROSPECTROPHOTOMETRY

In the study described here we have revealed an abnormal accumulation of porphyrin derivatives in the kidneys of Long-Evans Cinnamon (LEG) r ats, an animal model for human Wilson's disease, In addition, we have confirmed that the derivatives emitted red-orange Light in renal secti ons under UV excitation, This renal red-orange emission has previously been identified as luminescence from cuprous metallothioneins [Cu(I)- MTs], which also accumulate in both the kidneys and liver of LEC rats, In this study, we measured the emission spectra of the luminescence i n the kidneys using microspectrophotometry. The spectra of the renal r ed-orange emission resembled those of porphyrin derivatives rather tha n those of Cu(I)-MTs, We then extracted these derivatives horn the kid neys. An abundance of porphyrin derivatives was established. A signifi cant increase in the levels of the derivatives in the liver and urine of the LEC rats was also confirmed. These results provide evidence of a heme-metabolism abnormality in LEC rats. (C) 1998 Academic Press.