Spg7 - SPG7, paraplegin matrix AAA peptidase subunit

Predicted to enable several functions, including ATP binding activity; ATP hydrolysis activity; and peptidase activity. Acts upstream of or within anterograde axonal transport and mitochondrion organization. Located in mitochondrial inner membrane. Part of m-AAA complex. Is expressed in central nervous system; olfactory epithelium; and retina nuclear layer. Used to study hereditary spastic paraplegia 7. Human ortholog(s) of this gene implicated in hereditary spastic paraplegia 7. Orthologous to human SPG7 (SPG7 matrix AAA peptidase subunit, paraplegin). [provided by Alliance of Genome Resources, Apr 2022]