The channel hypothesis of Huntington's disease

Brain Research Bulletin

Volumn 56, Issue 3-4, 2001, Pages 281-284

  Kagan, Bruce L ^a   Hirakura, Yutaka ^b   Azimov, Rustam ^a,c   Azimova, Rushana ^a,c  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b WASEDA UNIVERSITY   (Japan)

^c Institute of Bioorganic Chemistry Uzbekistan Academy of Sciences   (Uzbekistan)

Author keywords

Amyloid; Apoptosis; Bilayers; Huntingtin; Membranes; Triplet repeat diseases

Indexed keywords

AMYLOID; HUNTINGTIN; ION CHANNEL; MUTANT PROTEIN; POLYGLUTAMINE;

ALZHEIMER DISEASE; CHANNEL GATING; CONFERENCE PAPER; CYTOTOXICITY; HUMAN; HUNTINGTON CHOREA; MEMBRANE POTENTIAL; PATHOLOGY; PRIORITY JOURNAL; TRINUCLEOTIDE REPEAT;

HUMANS; HUNTINGTON DISEASE; ION CHANNELS; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PEPTIDES; TRINUCLEOTIDE REPEATS;

EID: 0035504899     PISSN: 03619230     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0361-9230(01)00654-2     Document Type: Conference Paper

References (35)

1. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease
2. Alzheimer disease amyloid b protein forms-calcium channels in bilayers membranes; Blockage by tromethamine and aluminum
3. 2+ interaction with Alzheimer amyloid β protein calcium channels
4. Giant multilevel cation channels formed by Alzheimer disease amyloid beta-protein [A beta P-(1-40)] in bilayer membranes
5. Ion channels of different selectivity formed by transthyretin
6. Expansion of polyglutamine repeat in huntingtin leads to abnormal protein interactions involving calmodulin
7. Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses?
8. Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene
9. Synergistic activation by the glutamine-rich domains of human transcription factor Sp1
10. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation
11. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain
12. Theoretical Models of the ion channel structure of amyloid b-protein
13. Nuclear and neuropil aggregates in Huntington's disease; Relationship to neuropathology
14. Polyglutamine induced ion channels; A possible mechanism for the neurotoxicity of Huntington's and other CAG repeat diseases
15. Pore formation by B2-microglobulin; A mechanism for the pathogenesis of dialysis associated amyloidsis
16. Alzheimer amyloid Aβ 1-42 channels; Effects of solvent, pH, and Congo red
17. A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration
18. Alzheimer's disease amyloid-beta protein forms Zu(2+)- sensitive, cation selective channels across excised membrane patches from hypothalamic neurons
19. Cation selective channels formed by a C-terminal fragment of beta-amyloid precursor protein
20. Synthetic mammalian C-type natriuretic peptide forms large cation channels
21. Prion fragment PrP (106-126) forms distinct channel types
22. Properties of cytotoxic peptide-formed ion channels
23. Properties and modulation of alpha human atrial natriuretic peptide (a-hANP)-formed ion channels
24. Enhanced sensitivity to N-methyl-D-Aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease
25. The early cellular pathology of Huntington's disease
26. Channel formation by a neurotoxic prion protein fragment
27. Polyglutamines, nuclear inclusions and neurodegeneration
28. Pore formation by the cytotoxic islet amyloid peptide amylin
29. New tubular single-stranded helix of poly-L-amino acids suggested by molecular mechanics calculations. I. Homopolypeptides in isolated environments
30. Poly-L-Glutamine forms cation channels; Relevance to the pathogenesis of the polyglutamine diseases
31. Glutamine repeats as polar zippers; Their possible role in inherited neurodegenerative disease
32. Huntington-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo
33. Impaired synaptic plasticity in mice carrying the Huntington's disease mutation
34. Fresh and non-fibrillar amyloid beta protein (1-40) induced rapid cellular degeneration in aged human fibroblasts; Evidence for A beta-P channel mediated toxicity