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Literature DB >> 19591925

Impaired mitochondrial trafficking in Huntington's disease.

Abstract

Impaired mitochondrial function has been well documented in Huntington's disease. Mutant huntingtin is found to affect mitochondria via various mechanisms including the dysregulation of gene transcription and impairment of mitochondrial function or trafficking. The lengthy and highly branched neuronal processes constitute complex neural networks in which there is a large demand for mitochondria-generated energy. Thus, the impaired mitochondrial trafficking in neuronal cells may play an important role in the selective neuropathology of Huntington's disease. Here we discuss the evidence for the effect of the Huntington's disease protein huntingtin on the intracellular trafficking of mitochondria and the involvement of this defective trafficking in the pathogenesis of Huntington's disease.

Entities: Chemical Disease Gene Species

Mesh：

Substances：
Mitochondrial Proteins

Year: 2009 PMID： 19591925 PMCID： PMC2790549 DOI： 10.1016/j.bbadis.2009.06.008

Source DB: PubMed Journal: Biochim Biophys Acta ISSN： 0006-3002

49 in total

1. Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport.

Authors: Györgyi Szebenyi; Gerardo A Morfini; Alyssa Babcock; Milena Gould; Kimberly Selkoe; David L Stenoien; Maureen Young; Pieter W Faber; Marcy E MacDonald; Michael J McPhaul; Scott T Brady
Journal: Neuron Date: 2003-09-25 Impact factor: 17.173

2. Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

Authors: S J Tabrizi; M W Cleeter; J Xuereb; J W Taanman; J M Cooper; A H Schapira
Journal: Ann Neurol Date: 1999-01 Impact factor: 10.422

3. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.

Authors: Patrick Weydt; Victor V Pineda; Anne E Torrence; Randell T Libby; Terrence F Satterfield; Eduardo R Lazarowski; Merle L Gilbert; Gregory J Morton; Theodor K Bammler; Andrew D Strand; Libin Cui; Richard P Beyer; Courtney N Easley; Annette C Smith; Dimitri Krainc; Serge Luquet; Ian R Sweet; Michael W Schwartz; Albert R La Spada
Journal: Cell Metab Date: 2006-10-19 Impact factor: 27.287

4. Body weight and dietary factors in Huntington's disease patients compared with matched controls.

Authors: P R Sanberg; H C Fibiger; R F Mark
Journal: Med J Aust Date: 1981-04-18 Impact factor: 7.738

5. The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.

Authors: Erica Rockabrand; Natalia Slepko; Antonello Pantalone; Vidya N Nukala; Aleksey Kazantsev; J Lawrence Marsh; Patrick G Sullivan; Joan S Steffan; Stefano L Sensi; Leslie Michels Thompson
Journal: Hum Mol Genet Date: 2006-11-29 Impact factor: 6.150

Impaired mitochondrial trafficking in Huntington's disease.

1. Neuropathogenic forms of huntingtin and androgen receptor inhibit fast axonal transport.

2. Biochemical abnormalities and excitotoxicity in Huntington's disease brain.

3. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.

4. Body weight and dietary factors in Huntington's disease patients compared with matched controls.

5. The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis.

Review 6. Oxygen and ion concentrations in normoxic and hypoxic brain cells.

7. Huntingtin-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin.

8. Huntingtin phosphorylation acts as a molecular switch for anterograde/retrograde transport in neurons.

9. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines.

10. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons.

Review 1. Energy dysfunction in Huntington's disease: insights from PGC-1α, AMPK, and CKB.

Review 2. Differential vulnerability of neurons in Huntington's disease: the role of cell type-specific features.

Review 3. Energy deficit in Huntington disease: why it matters.

4. Altered lysosomal positioning affects lysosomal functions in a cellular model of Huntington's disease.

Review 5. Metabolism in HD: still a relevant mechanism?

6. Silver ion-induced mitochondrial dysfunction via a nonspecific pathway.

7. The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology.

8. Huntingtin interacts with the cue domain of gp78 and inhibits gp78 binding to ubiquitin and p97/VCP.

Review 9. Mitochondrial quality control and neurological disease: an emerging connection.

10. Neuroprotective effects of PPAR-γ agonist rosiglitazone in N171-82Q mouse model of Huntington's disease.