Steady-state levels of uroporphyrinogen decarboxylase mRNA in lymphoblastoid cell lines from patients with familial porphyria cutanea tarda and their relatives

Am J Hum Genet. 1988 Jun;42(6):847-53.

Abstract

Familial porphyria cutanea tarda (PCT) results from a generalized deficiency of uroporphyrinogen decarboxylase (URO-D) activity. The molecular defect responsible for this disorder has not been characterized. To determine whether decreased levels of URO-D mRNA are responsible for subnormal URO-D activity, steady-state levels of URO-D mRNA in lymphoblastoid cells were determined. Northern blots were hybridized with a URO-D cDNA probe and quantified by densitometry. No difference in the levels of URO-D mRNA was detected between affected individuals and their normal relatives. Thus, the deficiency of URO-D activity in two familial PCT pedigrees characterized here does not arise from a deficiency of URO-D mRNA.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Carboxy-Lyases / deficiency*
  • Cell Line
  • DNA / genetics
  • Humans
  • Lymphocytes / analysis
  • Lymphocytes / enzymology
  • Porphyrias / enzymology
  • Porphyrias / genetics*
  • RNA, Messenger / analysis
  • RNA, Messenger / genetics*
  • Skin Diseases / enzymology
  • Skin Diseases / genetics*
  • Uroporphyrinogen Decarboxylase / deficiency*
  • Uroporphyrinogen Decarboxylase / genetics

Substances

  • RNA, Messenger
  • DNA
  • Carboxy-Lyases
  • Uroporphyrinogen Decarboxylase