KEGG   NETWORK: N00989
Entry
N00989                      Network                                

Name
Mutation-caused aberrant Htt to electron transfer in Complex II
Definition
HTT* -| CxII -> QH2
  Expanded
3064v1 -| (6389+6390+6391+6392) -> C00390
Class
nt06418 Oxidative phosphorylation
nt06461 Huntington disease
Type
Variant
Pathway
hsa05016  Huntington disease
Disease
H00059  Huntington disease
Gene
3064  HTT; huntingtin
6389  SDHA; succinate dehydrogenase complex flavoprotein subunit A
6390  SDHB; succinate dehydrogenase complex iron sulfur subunit B
6391  SDHC; succinate dehydrogenase complex subunit C
6392  SDHD; succinate dehydrogenase complex subunit D
Variant
3064v1 (HTT*)  HTT mutation
Metabolite
C00390  Ubiquinol
Reference
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
DOI:10.1016/j.tins.2008.09.004
Reference
PMID:8602759
  Authors
Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AH
  Title
Mitochondrial defect in Huntington's disease caudate nucleus.
  Journal
Ann Neurol 39:385-9 (1996)
DOI:10.1002/ana.410390317
Reference
  Authors
Labbadia J, Morimoto RI
  Title
Huntington's disease: underlying molecular mechanisms and emerging concepts.
  Journal
Trends Biochem Sci 38:378-85 (2013)
DOI:10.1016/j.tibs.2013.05.003
Reference
  Authors
Mochel F, Haller RG
  Title
Energy deficit in Huntington disease: why it matters.
  Journal
J Clin Invest 121:493-9 (2011)
DOI:10.1172/JCI45691
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