KEGG   NETWORK: N00991
Entry
N00991                      Network                                

Name
Mutation-caused aberrant Htt to electron transfer in Complex III
Definition
HTT* -| CxIII -> CytC
  Expanded
3064v1 -| (7386+4519+1537+7384+7385+(440567,7388)+7381+27089+29796+10975) -> C00524
Class
nt06418 Oxidative phosphorylation
nt06461 Huntington disease
Type
Variant
Pathway
hsa05016  Huntington disease
Disease
H00059  Huntington disease
Gene
3064  HTT; huntingtin
7386  UQCRFS1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1
4519  CYTB; cytochrome b
1537  CYC1; cytochrome c1
7384  UQCRC1; ubiquinol-cytochrome c reductase core protein 1
7385  UQCRC2; ubiquinol-cytochrome c reductase core protein 2
440567  UQCRHL; ubiquinol-cytochrome c reductase hinge protein like
7388  UQCRH; ubiquinol-cytochrome c reductase hinge protein
7381  UQCRB; ubiquinol-cytochrome c reductase binding protein
27089  UQCRQ; ubiquinol-cytochrome c reductase complex III subunit VII
29796  UQCR10; ubiquinol-cytochrome c reductase, complex III subunit X
10975  UQCR11; ubiquinol-cytochrome c reductase, complex III subunit XI
Variant
3064v1 (HTT*)  HTT mutation
Metabolite
C00524  Cytochrome c
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
Chakraborty J, Rajamma U, Mohanakumar KP
  Title
A mitochondrial basis for Huntington's disease: therapeutic prospects.
  Journal
Mol Cell Biochem 389:277-91 (2014)
DOI:10.1007/s11010-013-1951-9
Reference
  Authors
Golpich M, Amini E, Mohamed Z, Azman Ali R, Mohamed Ibrahim N, Ahmadiani A
  Title
Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment.
  Journal
CNS Neurosci Ther 23:5-22 (2017)
DOI:10.1111/cns.12655
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