Mitochondrial import inner membrane translocase subunit Tim8 A (Deafness dystonia protein 1) (X-linked deafness dystonia protein)
1_MDSSS 6_ SSSAA 11_ GLGAV 16_ DPQLQ 21_ HFIEV 26_ ETQKQ 31_ RFQQL 36_ VHQMT 41_ ELCWE 46_ KCMDK 51_ PGPKL 56_ DSRAE 61_ ACFVN 66_ CVERF 71_ IDTSQ 76_ FILNR 81_ LEQTQ 86_ KSKPV 91_FSESL
1: Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development