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DNAJC19, a Mitochondrial Cochaperone Associated with Cardiomyopathy, Forms a Complex with Prohibitins to Regulate Cardiolipin Remodeling

Cell metabolism, 2014-07, Vol.20 (1), p.158-171

2014

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Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

Cell metabolism, 2014-03, Vol.19 (3), p.458-469

2014

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Loss of prohibitin membrane scaffolds impairs mitochondrial architecture and leads to tau hyperphosphorylation and neurodegeneration

PLoS genetics, 2012-11, Vol.8 (11), p.e1003021-e1003021

2012

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The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

Molecular cell, 2016-10, Vol.64 (1), p.148-162

2016

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Spastin binds to lipid droplets and affects lipid metabolism

PLoS genetics, 2015-04, Vol.11 (4), p.e1005149-e1005149

2015

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Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1

The Journal of cell biology, 2009-12, Vol.187 (7), p.1023-1036

2009

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The class 3 PI3K coordinates autophagy and mitochondrial lipid catabolism by controlling nuclear receptor PPARα

Nature communications, 2019-04, Vol.10 (1), p.1566-18, Article 1566

2019

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Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria

The Journal of cell biology, 2016-01, Vol.212 (2), p.157-166

2016

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Emerging roles of mitochondrial proteases in neurodegeneration

Biochimica et biophysica acta, 2010, Vol.1797 (1), p.1-10

2010

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Lipid Droplets in the Pathogenesis of Hereditary Spastic Paraplegia

Frontiers in molecular biosciences, 2021-05, Vol.8, p.673977-673977

2021

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CLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteins

The Journal of cell biology, 2014-10, Vol.207 (2), p.213-223

2014

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CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs

The Journal of cell biology, 2017-03, Vol.216 (3), p.675-693

2017

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Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases

PLoS genetics, 2011-10, Vol.7 (10), p.e1002325-e1002325

2011

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Loss of the mitochondrial i‐AAA protease YME1L leads to ocular dysfunction and spinal axonopathy

EMBO molecular medicine, 2019-01, Vol.11 (1), p.1-n/a

2019

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Mitochondrial quality control: a matter of life and death for neurons

The EMBO journal, 2012-03, Vol.31 (6), p.1336-1349

2012

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The m-AAA Protease Defective in Hereditary Spastic Paraplegia Controls Ribosome Assembly in Mitochondria

Cell, 2005-10, Vol.123 (2), p.277-289

2005

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The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying

PLoS genetics, 2016-12, Vol.12 (12), p.e1006463-e1006463

2016

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CLUH controls astrin-1 expression to couple mitochondrial metabolism to cell cycle progression

eLife, 2022-05, Vol.11

2022

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Variable and Tissue-Specific Subunit Composition of Mitochondrial m-AAA Protease Complexes Linked to Hereditary Spastic Paraplegia

Molecular and Cellular Biology, 2007-01, Vol.27 (2), p.758-767

2007

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Pleiotropic effects of spastin on neurite growth depending on expression levels

Journal of neurochemistry, 2009-03, Vol.108 (5), p.1277-1288

2009

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Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia

Trends in molecular medicine, 2006-06, Vol.12 (6), p.262-269

2006

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A cryptic promoter in the first exon of the SPG4 gene directs the synthesis of the 60-kDa spastin isoform

BMC biology, 2008-07, Vol.6 (1), p.31-31

2008

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A concert of RNA-binding proteins coordinates mitochondrial function

Critical reviews in biochemistry and molecular biology, 2018-11, Vol.53 (6), p.652-666

2018

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Alternative splicing of Spg7, a gene involved in hereditary spastic paraplegia, encodes a variant of paraplegin targeted to the endoplasmic reticulum

PloS one, 2012-05, Vol.7 (5), p.e36337-e36337

2012

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Genetic interaction between the m-AAA protease isoenzymes reveals novel roles in cerebellar degeneration

Human molecular genetics, 2009-06, Vol.18 (11), p.2001-2013

2009

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DNAJC19, a Mitochondrial Cochaperone Associated with Cardiomyopathy, Forms a Complex with Prohibitins to Regulate Cardiolipin Remodeling

Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

Loss of prohibitin membrane scaffolds impairs mitochondrial architecture and leads to tau hyperphosphorylation and neurodegeneration

The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

Spastin binds to lipid droplets and affects lipid metabolism

Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1

The class 3 PI3K coordinates autophagy and mitochondrial lipid catabolism by controlling nuclear receptor PPARα

Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria

Emerging roles of mitochondrial proteases in neurodegeneration

Lipid Droplets in the Pathogenesis of Hereditary Spastic Paraplegia

CLUH regulates mitochondrial biogenesis by binding mRNAs of nuclear-encoded mitochondrial proteins

CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs

Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases

Loss of the mitochondrial i‐AAA protease YME1L leads to ocular dysfunction and spinal axonopathy

Mitochondrial quality control: a matter of life and death for neurons

The m-AAA Protease Defective in Hereditary Spastic Paraplegia Controls Ribosome Assembly in Mitochondria

The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying

CLUH controls astrin-1 expression to couple mitochondrial metabolism to cell cycle progression

Variable and Tissue-Specific Subunit Composition of Mitochondrial m-AAA Protease Complexes Linked to Hereditary Spastic Paraplegia

Pleiotropic effects of spastin on neurite growth depending on expression levels

Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia

A cryptic promoter in the first exon of the SPG4 gene directs the synthesis of the 60-kDa spastin isoform

A concert of RNA-binding proteins coordinates mitochondrial function

Alternative splicing of Spg7, a gene involved in hereditary spastic paraplegia, encodes a variant of paraplegin targeted to the endoplasmic reticulum

Genetic interaction between the m-AAA protease isoenzymes reveals novel roles in cerebellar degeneration

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