Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex.

Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Research Abstract Details 

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Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Abstract Text:

Marcus P Cooper,Lishu Qu,Lindsay M Rohas,Jiandie Lin,Wenli Yang,Hediye Erdjument-Bromage,Paul Tempst,Bruce M Spiegelman,

Leigh syndrome French Canadian variant (LSFC) is an autosomal recessive neurodegenerative disorder due to mutation in the LRP130 (leucine-rich protein 130 kDa) gene. Unlike classic Leigh syndrome, the French Canadian variant spares the heart, skeletal muscle, and kidneys, but severely affects the liver. The precise role of LRP130 in cytochrome c oxidase deficiency and hepatic lactic acidosis that accompanies this disorder is unknown. We show here that LRP130 is a component of the PGC-1alpha (peroxisome proliferator-activated receptor coactivator 1-alpha) transcriptional coactivator holocomplex and regulates expression of PEPCK (phosphoenolpyruvate carboxykinase), G6P (glucose-6-phosphatase), and certain mitochondrial genes through PGC-1alpha. Reduction of LRP130 in fasted mice via adenoviral RNA interference (RNAi) vector blocks the induction of PEPCK and G6P, and blunts hepatic glucose output. LRP130 is also necessary for PGC-1alpha-dependent transcription of several mitochondrial genes in vivo. These data link LRP130 and PGC-1alpha to defective hepatic energy homeostasis in LSFC, and reveal a novel regulatory mechanism of glucose homeostasis.

Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Publishing Authors By Initials

MP Cooper,L Qu,LM Rohas,J Lin,W Yang,H Erdjument-Bromage,P Tempst,BM Spiegelman,

For similar proteins: dna-binding proteins: trans-activators research abstracts see: proteins: dna-binding proteins: trans-activators research

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Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Journal Published:

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

Journal: Genes & development

VOLUME: 20

Page Numbers: 2996-3009

Journal Abbreviation: Genes Dev.

ISSN: 0890-9369

DAY: 18

MONTH: 10

YEAR: 2006

Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 8711660

Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Keywords Mesh Terms:

KEYWORDS: Trans-Activators

MESH TERMS: metabolism

Chemical & Substance for Abstract: Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex. Information

Substance Name: Phosphoenolpyruvate Carboxykinase (ATP)

Registry Number: EC 4.1.1.49

Grant and Affiliation Information for Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex.

AFFILIATION: Dana-Farber Cancer Institute and the Department of Cell Biology, Harvard Medical School, Boston, MA 02115, USA.

Country: United States

AGENCY: United States NIDDK

GRANT: R01DK61562

ACRONYM: DK

MEDLINETA: Genes Dev

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