TIMM13 explained

Mitochondrial import inner membrane translocase subunit Tim13 is an enzyme that in humans is encoded by the TIMM13 gene.^{[1] [2] [3]}

Function

This gene encodes a translocase with similarity to yeast mitochondrial proteins that are involved in the import of metabolite transporters from the cytoplasm and into the mitochondrial inner membrane. The encoded protein and the TIMM8a protein form a 70 kDa complex in the intermembrane space. This gene is in a head-to-tail orientation with the gene for lamin B2.

Interactions

TIMM13 has been shown to interact with TIMM8A.^{[4] [5]}

Further reading

• Rehling P, Wiedemann N, Pfanner N, Truscott KN . The mitochondrial import machinery for preproteins . Critical Reviews in Biochemistry and Molecular Biology . 36 . 3 . 291–336 . 2001 . 11450972 . 10.1080/20014091074200 . 8758017 .
• Paschen SA, Neupert W . Protein import into mitochondria . IUBMB Life . 52 . 3–5 . 101–12 . 2002 . 11798021 . 10.1080/15216540152845894 . 31047665 . free .
• Neupert W, Brunner M . The protein import motor of mitochondria . Nature Reviews. Molecular Cell Biology . 3 . 8 . 555–65 . Aug 2002 . 12154367 . 10.1038/nrm878 . 5706589 .
• Jensen RE, Dunn CD . Protein import into and across the mitochondrial inner membrane: role of the TIM23 and TIM22 translocons . Biochimica et Biophysica Acta (BBA) - Molecular Cell Research . 1592 . 1 . 25–34 . Sep 2002 . 12191765 . 10.1016/S0167-4889(02)00261-6 .
• Bauer MF, Rothbauer U, Mühlenbein N, Smith RJ, Gerbitz K, Neupert W, Brunner M, Hofmann S . The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom . FEBS Letters . 464 . 1–2 . 41–7 . Dec 1999 . 10611480 . 10.1016/S0014-5793(99)01665-8 . 27484018 . free .
• Paschen SA, Rothbauer U, Káldi K, Bauer MF, Neupert W, Brunner M . The role of the TIM8-13 complex in the import of Tim23 into mitochondria . The EMBO Journal . 19 . 23 . 6392–400 . Dec 2000 . 11101512 . 305865 . 10.1093/emboj/19.23.6392 .
• Rothbauer U, Hofmann S, Mühlenbein N, Paschen SA, Gerbitz KD, Neupert W, Brunner M, Bauer MF . Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria . The Journal of Biological Chemistry . 276 . 40 . 37327–34 . Oct 2001 . 11489896 . 10.1074/jbc.M105313200 . free .
• Roesch K, Curran SP, Tranebjaerg L, Koehler CM . Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex . Human Molecular Genetics . 11 . 5 . 477–86 . Mar 2002 . 11875042 . 10.1093/hmg/11.5.477 . free .
• Chacinska A, Pfanner N, Meisinger C . How mitochondria import hydrophilic and hydrophobic proteins . Trends in Cell Biology . 12 . 7 . 299–303 . Jul 2002 . 12185844 . 10.1016/S0962-8924(02)02310-3 .
• Curran SP, Leuenberger D, Schmidt E, Koehler CM . The role of the Tim8p-Tim13p complex in a conserved import pathway for mitochondrial polytopic inner membrane proteins . The Journal of Cell Biology . 158 . 6 . 1017–27 . Sep 2002 . 12221072 . 2173223 . 10.1083/jcb.200205124 .
• Roesch K, Hynds PJ, Varga R, Tranebjaerg L, Koehler CM . The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex . Human Molecular Genetics . 13 . 18 . 2101–11 . Sep 2004 . 15254020 . 10.1093/hmg/ddh217 . free .
• Ewing RM, Chu P, Elisma F, Li H, Taylor P, Climie S, McBroom-Cerajewski L, Robinson MD, O'Connor L, Li M, Taylor R, Dharsee M, Ho Y, Heilbut A, Moore L, Zhang S, Ornatsky O, Bukhman YV, Ethier M, Sheng Y, Vasilescu J, Abu-Farha M, Lambert JP, Duewel HS, Stewart II, Kuehl B, Hogue K, Colwill K, Gladwish K, Muskat B, Kinach R, Adams SL, Moran MF, Morin GB, Topaloglou T, Figeys D . Large-scale mapping of human protein-protein interactions by mass spectrometry . Molecular Systems Biology . 3 . 1 . 89 . 2007 . 17353931 . 1847948 . 10.1038/msb4100134 .

Notes and References

1. Jin H, Kendall E, Freeman TC, Roberts RG, Vetrie DL . The human family of Deafness/Dystonia peptide (DDP) related mitochondrial import proteins . Genomics . 61 . 3 . 259–67 . Nov 1999 . 10552927 . 10.1006/geno.1999.5966 .
2. Gentle IE, Perry AJ, Alcock FH, Likić VA, Dolezal P, Ng ET, Purcell AW, McConnville M, Naderer T, Chanez AL, Charrière F, Aschinger C, Schneider A, Tokatlidis K, Lithgow T . Conserved motifs reveal details of ancestry and structure in the small TIM chaperones of the mitochondrial intermembrane space . Molecular Biology and Evolution . 24 . 5 . 1149–60 . May 2007 . 17329230 . 10.1093/molbev/msm031 . free .
3. Web site: Entrez Gene: TIMM13 translocase of inner mitochondrial membrane 13 homolog (yeast).
4. Ewing RM, Chu P, Elisma F, Li H, Taylor P, Climie S, McBroom-Cerajewski L, Robinson MD, O'Connor L, Li M, Taylor R, Dharsee M, Ho Y, Heilbut A, Moore L, Zhang S, Ornatsky O, Bukhman YV, Ethier M, Sheng Y, Vasilescu J, Abu-Farha M, Lambert JP, Duewel HS, Stewart II, Kuehl B, Hogue K, Colwill K, Gladwish K, Muskat B, Kinach R, Adams SL, Moran MF, Morin GB, Topaloglou T, Figeys D . Large-scale mapping of human protein-protein interactions by mass spectrometry . Molecular Systems Biology . 3 . 1 . 89 . 2007 . 17353931 . 1847948 . 10.1038/msb4100134 .
5. Roesch K, Curran SP, Tranebjaerg L, Koehler CM . Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex . Human Molecular Genetics . 11 . 5 . 477–86 . Mar 2002 . 11875042 . 10.1093/hmg/11.5.477 . free .