|1.||Langer, Thomas: 11 articles (10/2011 - 11/2003)|
|2.||Rugarli, Elena I: 6 articles (10/2011 - 10/2005)|
|3.||Koppen, Mirko: 4 articles (10/2009 - 11/2003)|
|4.||Tatsuta, Takashi: 3 articles (02/2013 - 01/2007)|
|5.||Bonn, Florian: 3 articles (10/2011 - 10/2009)|
|6.||Ehses, Sarah: 3 articles (12/2009 - 10/2005)|
|7.||Martinelli, Paola: 2 articles (10/2011 - 01/2010)|
|8.||Augustin, Steffen: 2 articles (02/2011 - 01/2007)|
|9.||Bernacchia, Andrea: 2 articles (12/2009 - 10/2005)|
|10.||Nolden, Mark: 2 articles (01/2007 - 10/2005)|
|1.||Hereditary Spastic Paraplegia
05/01/2010 - "An autosomal recessive form of hereditary spastic paraplegia (AR-HSP) is primarily caused by mutations in the SPG7 gene, which codes for paraplegin, a subunit of the hetero-oligomeric m-AAA protease in mitochondria. "
01/01/2009 - "Paraplegin is an m-AAA protease of the mitochondrial inner membrane that is linked to hereditary spastic paraplegias. "
01/01/2007 - "Mutations in the m-AAA protease subunit paraplegin cause axonal degeneration in hereditary spastic paraplegia (HSP), but the basis for the unexpected tissue specificity is not understood. "
01/01/2007 - "Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia."
06/01/2006 - "Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia."
|2.||Neurodegenerative Diseases (Neurodegenerative Disease)
10/01/2009 - "These findings might be of relevance for the pathogenesis of neurodegenerative disorders associated with mutations in different m-AAA protease subunits."
02/11/2011 - "Mutations in subunits of mitochondrial m-AAA proteases have been associated with different neurodegenerative disorders in human, raising questions on the functional differences between homo- and hetero-oligomeric AAA proteases. "
01/01/2010 - "Mitochondrial proteases may be directly involved in neurodegenerative diseases, as recently shown for the m-AAA protease, or may regulate crucial mitochondrial molecules, such as OPA1, which in turn is implicated in human disease. "
12/28/2009 - "Our findings link distinct peptidases to constitutive and induced OPA1 processing and shed new light on the pathogenesis of neurodegenerative disorders associated with mutations in m-AAA protease subunits."
07/29/2014 - "The p97 AAA (ATPase associated with diverse cellular activities), also called VCP (valosin-containing protein), is an important therapeutic target for cancer and neurodegenerative diseases. "
02/15/2013 - "Dislocation by the m-AAA protease increases the threshold hydrophobicity for retention of transmembrane helices in the inner membrane of yeast mitochondria."
01/24/2007 - "Decreasing the hydrophobicity of the Ccp1 transmembrane segment facilitates its dislocation from the membrane and renders rhomboid cleavage m-AAA protease-independent. "
01/24/2007 - "m-AAA protease-driven membrane dislocation allows intramembrane cleavage by rhomboid in mitochondria."
07/01/2004 - "We therefore propose that transmembrane segments of m-AAA protease subunits have a direct role in the dislocation of membrane-embedded substrates."
01/24/2007 - "The m-AAA protease mediates the ATP-dependent membrane dislocation of Ccp1 independent of its proteolytic activity. "
|4.||Cerebellar Ataxia (Dysmetria)
01/01/2015 - "OPA1 cleavage is regulated by two m-AAA proteases, SPG7 and AFG3L2, which are, respectively involved in Spastic Paraplegia 7 and Spino-Cerebellar Ataxia 28. "
10/01/2010 - "Spinocerebellar ataxia type 28 is an autosomal dominant form of cerebellar ataxia (ADCA) caused by mutations in AFG3L2, a gene that encodes a subunit of the mitochondrial m-AAA protease. "
|5.||Spinocerebellar Ataxias (Spinocerebellar Ataxia)
|1.||Peptide Hydrolases (Proteases)
|2.||Adenosine Triphosphate (ATP)