Inactivation of the mitochondrial protease Afg3l2 results in severely diminished respiratory chain activity and widespread defects in mitochondrial gene expression

Autophagy accounts for approximately one-third of mitochondrial protein turnover and is protein selective

(2019) Evelyn S. Vincow et al.   Autophagy

m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration

(2018) Maria Patron et al.   CELL RESEARCH

Mitophagy and Quality Control Mechanisms in Mitochondrial Maintenance

(2018) Sarah Pickles et al.   CURRENT BIOLOGY

Mitochondrial Proteostasis in the Context of Cellular and Organismal Health and Aging

(2018) Erica A Moehle et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration

(2018) Gautam Pareek et al.   Cell Death & Disease

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

The Mitochondrial Basis of Aging

(2016) Nuo Sun et al.   MOLECULAR CELL

Loss of Drosophila i-AAA protease, dYME1L, causes abnormal mitochondria and apoptotic degeneration

(2015) Y Qi et al.   CELL DEATH AND DIFFERENTIATION

New roles for mitochondrial proteases in health, ageing and disease

(2015) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Highly Specific and Efficient CRISPR/Cas9-Catalyzed Homology-Directed Repair inDrosophila

(2014) Scott J. Gratz et al.   GENETICS

PINK1-Parkin Pathway Activity Is Regulated by Degradation of PINK1 in the Mitochondrial Matrix

(2014) Ruth E. Thomas et al.   PLoS Genetics

Mitochondrial AAA proteases — Towards a molecular understanding of membrane-bound proteolytic machines

(2011) Florian Gerdes et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Haploinsufficiency of AFG3L2, the Gene Responsible for Spinocerebellar Ataxia Type 28, Causes Mitochondria-Mediated Purkinje Cell Dark Degeneration

(2009) F. Maltecca et al.   JOURNAL OF NEUROSCIENCE