above the normal limit, was obtained. The Aminolevulinic acid, porphobilinogen, and other patient, a 54-year-old male with Hgb SC disease and infected leg ulcer, showed 9 total porphyrin values from 4 to 149 pg/24 hr and only one value, 208 pg/24 hr above the normal limit of 180 pg/24 hr.Discussion. Our findings reveal a rather consistent moderate, occasionally marked increase of urinary copro-, total, and uroporphyrins-in this order-and to a slight extent of porphyrin precursors ALA, PBG, and AA during painful and febrile sickle cell crises. Although the number of cases is small, it is our impression that total urinary porphyrin excretion generally parallels the severity of clinical symptomatology and is normal during recovery and the steady state.Of considerable interest is the close resemblance of prominent clinical features in sickle cell crisis and acute porphyria, particularly evident in cases with episodes of excruciating abdominal pain which in both conditions has led to unnecessary laparotomies (9,10,11) . Besides increase of urinary porphyrins there is evidence of vasoconstriction in both diseases(9,10,12,13).Summary. Porphyrin excretion in urine was studied in 18 patients with sickle cell disease, including multiple observations in 6 patients during 7 typical painful and febrile crises. With few exceptions there was moderate to marked elevation of copro-, total, and uroporphyrins at time of pain and fever. aminoacetone during crises were within range of normal variation or only slightly increased. Following subsidence of pain and fever, total porphyrin values returned toward normal. Similarities between clinical features of painful sickle cell crisis and acute porphyria are pointed out.

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Huntingtin was localized by using a series of antibodies that detected di¡erent areas of the protein from the immediate N-terminus to the C-terminal region of the protein. The more C-terminal antibodies gave a cytoplasmic localization in neurons of the brain in controls and cases of Huntington's disease (HD). The N-terminal antibody, however, gave a distinctive pattern of immunoreactivity in the HD brain, with marked staining of axon tracts and white matter and the detection of densely staining intranuclear inclusions. This implies some processing di¡erences between mutated and normal huntingtin. We have also localized two interacting proteins, cystathionine b-synthase and the nuclear receptor co-repressor (N-CoR), in brain. Cystathionine b-synthase was not relocalized in HD brain, but the N-CoR was excluded from neuronal nuclei in HD brain, and a further protein that exists in the same repression complex, mSin3, was similarly excluded. We conclude that the co-repressor might have a part in HD pathology.