Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences.

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Research Abstract Details 

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Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Abstract Text:

Tamara Milakovic,Rodrigo A Quintanilla,Gail V W Johnson,

Huntington disease (HD) is caused by a pathological elongation of CAG repeats in the huntingtin protein gene and is characterized by atrophy and neuronal loss primarily in the striatum. Mitochondrial dysfunction and impaired Ca2+ homeostasis in HD have been suggested previously. Here, we elucidate the effects of Ca2+ on mitochondria from the wild type (STHdhQ7/Q7) and mutant (STHdhQ111/Q111) huntingtin-expressing cells of striatal origin. When treated with increasing Ca2+ concentrations, mitochondria from mutant huntingtin-expressing cells showed enhanced sensitivity to Ca2+, as they were more sensitive to Ca2+-induced decreases in state 3 respiration and DeltaPsim, than mitochondria from wild type cells. Further, mutant huntingtin-expressing cells had a reduced mitochondrial Ca2+ uptake capacity in comparison with wild type cells. Decreases in state 3 respiration were associated with increased mitochondrial membrane permeability. The DeltaPsim defect was attenuated in the presence of ADP and the decreases in Ca2+ uptake capacity were abolished in the presence of Permeability Transition Pore (PTP) inhibitors. These findings clearly indicate that mutant huntingtin-expressing cells have mitochondrial Ca2+ handling defects that result in respiratory deficits and that the increased sensitivity to Ca2+ induced mitochondrial permeabilization maybe a contributing mechanism to the mitochondrial dysfunction in HD.

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Publishing Authors By Initials

T Milakovic,RA Quintanilla,GV Johnson,

For similar proteins: nuclear proteins research abstracts see: proteins: nuclear proteins research

PUBMED ID PMID:

MEDLINE DATE:

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: The Journal of biological chemistry

VOLUME: 281

Page Numbers: 34785-95

Journal Abbreviation: J. Biol. Chem.

ISSN: 0021-9258

DAY: 13

MONTH: 09

YEAR: 2006

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 2985121

Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Keywords Mesh Terms:

KEYWORDS: Nuclear Proteins

MESH TERMS: metabolism

Chemical & Substance for Abstract: Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. Information

Substance Name: Calcium

Registry Number: 7440-70-2

Grant and Affiliation Information for Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences.

AFFILIATION: Department of Psychiatry, University of Alabama at Birmingham, Birmingham, Alabama 35294-0017, USA.

Country: United States

AGENCY: United States NINDS

GRANT: NS041744

ACRONYM: NS

MEDLINETA: J Biol Chem

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