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Background  The  m -AAA ( A TPases  A ssociated with a variety of cellular  A ctivities) is an evolutionary conserved metalloprotease complex located in the internal mitochondrial membrane. In the mouse, it is a hetero-oligomer variably formed by the  Spg7 ,  Afg3l1 , and  Afg3l2  encoded proteins, or a homo-oligomer formed by either Afg3l1 or Afg3l2. In humans,  AFG3L2  and  SPG7  genes are conserved, whereas  AFG3L1  became a pseudogene. Both  AFG3L2  and  SPG7  are involved in a neurodegenerative disease, namely the autosomal dominant spinocerebellar ataxia SCA28 and a recessive form of spastic paraplegia, respectively.    Results  Using quantitative RT-PCR, we measured the expression levels of  Spg7 ,  Afg3l1 , and  Afg3l2  in the mouse brain. In all regions  Afg3l2  is the most abundant transcript, followed by  Spg7 , and  Afg3l1 , with a ratio of approximately 5:3:1 in whole-brain mRNA. Using  in-situ  hybridization, we showed that  Spg7 ,  Afg3l1  and  Afg3l2  have a similar cellular pattern of expression, with high levels in mitral cells, Purkinje cells, deep cerebellar nuclei cells, neocortical and hippocampal pyramidal neurons, and brainstem motor neurons. However, in some neuronal types, differences in the level of expression of these genes were present, suggesting distinct degrees of contribution of their proteins.    Conclusions  Neurons involved in SCA28 and hereditary spastic paraplegia display high levels of expression, but similar or even higher expression is also present in other types of neurons, not involved in these diseases, suggesting that the selective cell sensitivity should be attributed to other, still unknown, mechanisms.

Mouse brain expression patterns of Spg7, Afg3l1, and Afg3l2 transcripts, encoding for the mitochondrial m-AAA protease