The function of mitochondria depends on ubiquitously expressed and evolutionary conserved m-AAA proteases in the inner membrane. These ATP-dependent peptidases form hexameric complexes built up of homologous subunits. AFG3L2 subunits assemble either into homo-oligomeric isoenzymes or with SPG7 (paraplegin) subunits into hetero-oligomeric proteolytic complexes. Mutations in AFG3L2 are associated with dominant spinocerebellar ataxia (SCA28) characterized by the loss of Purkinje cells, whereas mutations in SPG7 cause a recessive form of hereditary spastic paraplegia (HSP7) with motor neurons of the cortico-spinal tract being predominantly affected. Pleiotropic functions have been assigned to m-AAA proteases, which act as quality control and re...
Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disease caused by mutations of the mit...
To ensure the removal of excess and non-assembled proteins, mitochondria require a protein quality c...
The m-AAA protease, an ATP-dependent proteolytic complex in the inner mitochondrial membrane, contro...
The function of mitochondria depends on ubiquitously expressed and evolutionary conserved m-AAA prot...
Mutations in subunits of mitochondrial m-AAA proteases in the inner membrane cause neurodegeneration...
The m-AAA protease preserves proteostasis of the inner mitochondrial membrane. It ensures a function...
Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disorder characterized by unbalanced s...
Mutations in the AFG3L2 gene have been linked to spinocerebellar ataxia type 28 and spastic ataxia-n...
Mutations in the AFG3L2 gene have been linked to spinocerebellar ataxia type 28 and spastic ataxia-n...
The m-AAA protease subunit AFG(3)L(2) is involved in degradation and processing of substrates in the...
Autosomal dominant spinocerebellar ataxias (SCAs) are genetically heterogeneous neurological disorde...
AbstractFine tuning of integrated mitochondrial functions is essential in neurons and rationalizes w...
Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that i...
m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which a...
m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which a...
Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disease caused by mutations of the mit...
To ensure the removal of excess and non-assembled proteins, mitochondria require a protein quality c...
The m-AAA protease, an ATP-dependent proteolytic complex in the inner mitochondrial membrane, contro...
The function of mitochondria depends on ubiquitously expressed and evolutionary conserved m-AAA prot...
Mutations in subunits of mitochondrial m-AAA proteases in the inner membrane cause neurodegeneration...
The m-AAA protease preserves proteostasis of the inner mitochondrial membrane. It ensures a function...
Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disorder characterized by unbalanced s...
Mutations in the AFG3L2 gene have been linked to spinocerebellar ataxia type 28 and spastic ataxia-n...
Mutations in the AFG3L2 gene have been linked to spinocerebellar ataxia type 28 and spastic ataxia-n...
The m-AAA protease subunit AFG(3)L(2) is involved in degradation and processing of substrates in the...
Autosomal dominant spinocerebellar ataxias (SCAs) are genetically heterogeneous neurological disorde...
AbstractFine tuning of integrated mitochondrial functions is essential in neurons and rationalizes w...
Hereditary spastic paraplegia (HSP) is a genetically heterogeneous neurodegenerative disorder that i...
m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which a...
m-AAA proteases are ATP-dependent proteolytic machines in the inner membrane of mitochondria which a...
Spinocerebellar ataxia type 28 (SCA28) is a neurodegenerative disease caused by mutations of the mit...
To ensure the removal of excess and non-assembled proteins, mitochondria require a protein quality c...
The m-AAA protease, an ATP-dependent proteolytic complex in the inner mitochondrial membrane, contro...