Regulation of thioredoxin reductase by calcium in Hermansky-Pudlak syndrome.

Cell cultures of keratinocytes, established from four Hermansky-Pudlak syndrome (HPS) homozygotes yielded low membrane-associated thioredoxin reductase activities compared with normal healthy adult controls. This low activity has been shown to be caused by a special sensitivity of the enzyme to calcium. 45Calcium has been used to compare the kinetics for the transport and bioaccumulation of this regulatory cation in keratinocyte cultures of a kindred with HPS (i.e., one HPS homozygote, one HPS obligate heterozygote, one normal family member, and healthy adult controls). The results show that both HPS-homozygous and -heterozygous cells bind more extracellular calcium than noncarriers of this genetic defect, and HPS homozygous cells appear to have a defective calcium transport system.
AuthorsK U Schallreuter, M R Pittelkow
JournalArchives of dermatological research (Arch Dermatol Res) Vol. 281 Issue 1 Pg. 40-4 ( 1989) ISSN: 0340-3696 [Print] GERMANY, WEST
PMID2730141 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • NADH, NADPH Oxidoreductases
  • Thioredoxin-Disulfide Reductase
  • Calcium
  • Absorption
  • Adult
  • Albinism
  • Biological Transport
  • Blood Platelet Disorders
  • Calcium (analysis, pharmacokinetics)
  • Female
  • Humans
  • In Vitro Techniques
  • Male
  • NADH, NADPH Oxidoreductases (metabolism)
  • Skin Diseases (metabolism)
  • Syndrome
  • Thioredoxin-Disulfide Reductase (metabolism)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!

Choose Username:
Verify Password: