Linked Life Data

12175877

Source:http://linkedlifedata.com/resource/pubmed/id/12175877

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3-5
pubmed:dateCreated
2002-8-14
pubmed:databankReference
pubmed:abstractText
The neuronal storage diseases are a rare group of disorders with profound clinical consequences including severe mental retardation and death in early childhood. A subset of these disorders, those with elevated levels of GM2 ganglioside, are further characterized by the reinitiation of primary dendrites on mature cortical neurons. These ectopic dendrites are unusual as primary dendrite initiation is normally confined to a narrow developmental window. Thus, ectopic dendritogenesis appears to be a recapitulation of the normal developmental program temporally displaced. Consequently, understanding ectopic dendritogenesis should offer insights into both the pathogenesis of the neuronal storage diseases as well as mechanisms of normal CNS development. Using a feline model of GM2 gangliosidosis, we compared patterns of gene expression in normal newborn and mature diseased animals (both undergoing active primary dendritogenesis) with normal, mature controls (where primary dendritogenesis has ceased). From this work, we have identified two genes that appear to function in primary dendrite initiation. One, tomoregulin, is an integral membrane protein with both EGF- and follistatin-like motifs in its extracellular domain. The second, Tristanin, is a member of the positive regulatory domain (PRD) family of a zinc-finger transcription factors. Both genes are up regulated in the disease state, and both show a shift in their intracellular location to the nucleus in diseased animals that is not observed in age matched controls. In normal mouse brain, tomoregulin and Tristanin reveal developmental patterns consistent with a role in dendrite initiation and show changes in subcellular localization similar to that observed in the cat.
pubmed:commentsCorrections
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0736-5748
pubmed:author
pubmed:issnType
Print
pubmed:volume
20
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
373-89
pubmed:dateRevised
2010-11-18
pubmed:meshHeading
pubmed-meshheading:12175877-Animals, pubmed-meshheading:12175877-Animals, Newborn, pubmed-meshheading:12175877-Cats, pubmed-meshheading:12175877-Cell Differentiation, pubmed-meshheading:12175877-Cells, Cultured, pubmed-meshheading:12175877-Cerebral Cortex, pubmed-meshheading:12175877-DNA, Complementary, pubmed-meshheading:12175877-DNA-Binding Proteins, pubmed-meshheading:12175877-Dendrites, pubmed-meshheading:12175877-Disease Models, Animal, pubmed-meshheading:12175877-Fetus, pubmed-meshheading:12175877-Gangliosidoses, GM2, pubmed-meshheading:12175877-Genetic Testing, pubmed-meshheading:12175877-Immunohistochemistry, pubmed-meshheading:12175877-Membrane Proteins, pubmed-meshheading:12175877-Mice, pubmed-meshheading:12175877-Mice, Inbred C57BL, pubmed-meshheading:12175877-Molecular Sequence Data, pubmed-meshheading:12175877-Neoplasm Proteins, pubmed-meshheading:12175877-Pyramidal Cells, pubmed-meshheading:12175877-RNA, Messenger, pubmed-meshheading:12175877-Sequence Homology, Amino Acid, pubmed-meshheading:12175877-Sequence Homology, Nucleic Acid, pubmed-meshheading:12175877-Transcription Factors
pubmed:articleTitle
Ectopic dendrite initiation: CNS pathogenesis as a model of CNS development.
pubmed:affiliation
Department of Neuroscience, Albert Einstein College of Medicine, Kennedy Center, Bronx, NY 10461, USA. dsiegel@aecom.yu.edu
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't