Linked Life Data

12196178

Source:http://linkedlifedata.com/resource/pubmed/id/12196178

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
2002-8-28
pubmed:abstractText
Cellular iron homoeostasis is maintained by iron sensor proteins known as iron-regulatory proteins (IRPs), which act post-transcriptionally by binding RNA stem-loop structures, termed iron-responsive elements (IREs), present on the mRNAs of proteins involved in iron storage, utilization and transport. IRP1 is a bifunctional protein that can act either as a cytoplasmic aconitase or as an IRE-binding protein. The RNA-binding activity of IRP1 is regulated post-translationally by the insertion or extrusion of a 4Fe-4S cluster, without changes in the levels of protein. In hereditary haemochromatosis (HH) accumulation of iron in parenchymal tissues, including the liver, occurs, possibly through dysfunctional IRP1. Investigation of IRP1 expression in liver biopsies from HH patients showed that the protein is completely absent or markedly reduced in heavily iron-loaded HH patients. Real-time PCR was then conducted in an attempt to investigate the mRNA levels and establish the underlying mechanism behind the disappearing act of IRP1. The two possibilities are: transcriptional regulation (through the inhibition of transcription) or post-transcriptional regulation (either through increased turnover of protein or inhibition of translation) of IRP1. Preliminary data suggest that transcription of IRP1 is not affected by chronic iron overload, and down-regulation may be attributable instead to degradation of the protein.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0300-5127
pubmed:author
pubmed:issnType
Print
pubmed:volume
30
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
726-8
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
2002
pubmed:articleTitle
Down-regulation of liver iron-regulatory protein 1 in haemochromatosis.
pubmed:affiliation
Institute of Liver Studies, Guy's, King's and St Thomas' School of Medicine, King's College Hospital, Bessemer Road, London SE5 9PJ, UK. maria.neonaki@kcl.ac.uk
pubmed:publicationType
Journal Article