Transmissible and genetic prion diseases share a common pathway of neurodegeneration
Article Abstract:
There seem to be causal relationships between accumulation of PrPSc, an abnormally folded isoform of the prion protein (PrP), the events of formation and metabolism of CtmPrP, a transmembrane form of PrP, and the development of neurodegenerative disease. It has been established that the effectiveness of accumulated PrPSc in sparking off neurodegenerative disease is determined by the predilection of host-encoded PrP to be made into the CtmPrP form. It appears that there are three successive stages in the pathogenesis of prion diseases.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1999
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Molecular mechanism for an inherited cardiac arrhythmia
Article Abstract:
Studies of the mutation in the gene encoding the human heart voltage-gated sodium-channel alpha-subunit (SCN5A) showed a sustained inward current during membrane depolarization in the mutant channel. The single-channel recordings revealed that mutant channels fluctuate between normal and non-inactivating gating modes. The sustained inward sodium current results in prolongation of cardiac action potentials, thus explaining one molecular mechanism for congenital long-QT syndrome.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1995
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Epithelial sodium channel related to proteins involved in neurodegeneration
Article Abstract:
A DNA has been isolated from rat epithelial cells which codes a protein which affects sodium channels. The protein has 698 amino acids and probably has two or more transmembrane segments. The gene is similar to mec-4 and deg-1 which can be mutated to participate in neurodegeneration. All three genes are probably members of the same family which codes for cation channels.
Publication Name: Nature
Subject: Zoology and wildlife conservation
ISSN: 0028-0836
Year: 1993
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