This family has 26 members: 2 Arabidopsis thaliana, 2 Caenorhabditis elegans, 3 Danio rerio, 2 Drosophila melanogaster, 1 Gallus gallus, 3 Homo sapiens, 5 Mus musculus, 4 Rattus norvegicus, 2 Saccharomyces cerevisiae, 2 Schizosaccharomyces pombe.
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| Organism | Protein (Synonyms) | Description | AmiGO |
|---|---|---|---|
| A. thaliana | NCBI:NP_564780.1 · TAIR:locus:2200918 (T25B24.8 · T25B24_8 · TIM13 · AT1G61570) | TRANSLOCASE OF THE INNER MITOCHONDRIAL MEMBRANE 13 |  |
| A. thaliana | NCBI:NP_199894.1 · TAIR:locus:2157931 (TIM8 · K7B16_3 · K7B16.3 · AT5G50810) | | |
| C. elegans | WB:WBGene00006574 (tin-13 · DY3.1) · UniProtKB:O45319 | | |
| C. elegans | WB:WBGene00000941 (Y39A3CR.4 · ddp-1) · UniProtKB:Q9N408 | | |
| D. rerio | ENSEMBL:ENSDARP00000072577 | ||
| D. rerio | ZFIN:ZDB-GENE-040718-167 (zgc:92895) · UniProtKB:Q6DGJ3 | | |
| D. rerio | UniProtKB:Q6DEM5 · ZFIN:ZDB-GENE-040801-158 (timm8a) | translocase of inner mitochondrial membrane 8 homolog A (yeast) | |
| D. melanogaster | FB:FBgn0036204 (CG11611 · Tim13) · UniProtKB:Q9VTN3 | | |
| D. melanogaster | FB:FBgn0027359 (BcDNA:SD06593 · CG1728 · Tim8) · UniProtKB:Q9Y1A3 | | |
| G. gallus | NCBI:XP_420185 · ENTREZ:422190 | ||
| H. sapiens | ENSEMBL:ENSG00000099800 · UniProtKB:Q9Y5L4 (IPI00001589 · Q9Y5L4 · TIMM13B · TIM13_HUMAN · TIMM13A · TIM13B · TIMM13) | Mitochondrial import inner membrane translocase subunit Tim13 | |
| H. sapiens | ENSEMBL:ENSG00000126953 · UniProtKB:O60220 (DDP1 · TIMM8A · TIM8A · DDP · O60220 · TIM8A_HUMAN · IPI00028376) | Mitochondrial import inner membrane translocase subunit Tim8 A | |
| H. sapiens | ENSEMBL:ENSG00000150779 · UniProtKB:Q9Y5J9 (DDPL · TIM8B · DDP2 · IPI00001546 · TIM8B_HUMAN · TIMM8B · Q9Y5J9) | Mitochondrial import inner membrane translocase subunit Tim8 B | |
| M. musculus | NCBI:XP_001473691 · MGI:MGI:3704349 | ||
| M. musculus | NCBI:NP_038923 · MGI:MGI:1353432 (Timm13 · Tim9 · D10Ertd378e · Timm9) | translocase of inner mitochondrial membrane 13 homolog (yeast) | |
| M. musculus | UniProtKB:Q4FZG7 · MGI:MGI:3615492 (Timm8a2) | translocase of inner mitochondrial membrane 8 homolog a2 (yeast) | |
| M. musculus | UniProtKB:Q9WVA2 · MGI:MGI:1353433 (Fci-12 · Tim8a · DXHXS1274E · Timm8a1 · Ddp1) | translocase of inner mitochondrial membrane 8 homolog a1 (yeast) | |
| M. musculus | UniProtKB:P62077 · MGI:MGI:1353424 (Tim8b · Timm8b) | translocase of inner mitochondrial membrane 8 homolog b (yeast) | |
| R. norvegicus | UniProtKB:P62076 · RGD:628845 (Timm13) | translocase of inner mitochondrial membrane 13 homolog (yeast) | |
| R. norvegicus | UniProtKB:Q9WVA1 · RGD:621801 (Timm8a1) | translocase of inner mitochondrial membrane 8 homolog a1 (yeast) | |
| R. norvegicus | UniProtKB:P62078 · RGD:619840 (Timm8b) | translocase of inner mitochondrial membrane 8 homolog b (yeast) | |
| R. norvegicus | NCBI:XP_001058904 · RGD:1588172 | ||
| S. cerevisiae | UniProtKB:P53299 · SGD:S000003413 (YGR181W · TIM13) | Mitochondrial intermembrane space protein, forms a complex with Tim8p that mediates import and insertion of a subset of polytopic inner membrane proteins | |
| S. cerevisiae | UniProtKB:P57744 · SGD:S000007348 (TIM8 · YJR135W-A) | Mitochondrial intermembrane space protein mediating import and insertion of polytopic inner membrane proteins | |
| S. pombe | UniProtKB:Q10481 · GeneDB_Spombe:SPAC17C9.09c (SPAC17C9.09c · tim13) | TIM22 inner membrane protein import complex subunit Tim13 | |
| S. pombe | UniProtKB:Q09783 · GeneDB_Spombe:SPAC13G6.04 (tim8 · SPAC13G6.04) | TIM22 inner membrane protein import complex subunit Tim8 | |
| Protein | Publication | Curator Notes | |
|---|---|---|---|
| UniProtKB:P53299 · SGD:S000003413 |  11489896 Rothbauer U, et al. Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem. 2001 Oct 5;276(40):37327-34. | The H. sapiens protein Q9Y5L4 complements a homologous mutation in S. cerevisiae. | |
| UniProtKB:P57744 · SGD:S000007348 | 11489896 Rothbauer U, et al. Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem. 2001 Oct 5;276(40):37327-34. | The H. sapiens protein O60220 complements a homologous mutation in S. cerevisiae. | |
| UniProtKB:P57744 · SGD:S000007348 | 11956200 Hofmann S, et al. The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. J Biol Chem. 2002 Jun 28;277(26):23287-93. | The H. sapiens protein O60220 complements a homologous mutation in S. cerevisiae. | |
| UniProtKB:P53299 · SGD:S000003413 | 11956200 Hofmann S, et al. The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. J Biol Chem. 2002 Jun 28;277(26):23287-93. | The H. sapiens protein Q9Y5L4 complements a homologous mutation in S. cerevisiae. |
| Description | Suffix |
|---|---|
| Sequences in this family | .fasta |
| mafft aligned Fasta file | .afasta |
| phyml newick file | .newick |
| Notung rooted & rearranged newick file | .newick.rooting.0.rearrange.0 |
| Notung Homolog Table | .newick.rooting.0.rearrange.0.homologs.csv |
| OMIM (2) | |
|---|---|
| ENSEMBL:ENSG00000126953 · UniProtKB:O60220 | #304700 MOHR-TRANEBJAERG SYNDROME; MTS;;DYSTONIA-DEAFNESS SYNDROME; DDS;;DEAFNESS-DYSTONIA-OPTIC ATROPHY SYNDROME; DDP;;DEAFNESS SYNDROME, PROGRESSIVE, WITH BLINDNESS, DYSTONIA, FRACTURES,AND MENTAL DEFICIENCY |
| UniProtKB:P53299 · SGD:S000003413 | #311150 OPTICOACOUSTIC NERVE ATROPHY WITH DEMENTIA;;JENSEN SYNDROME |
| SGD Disease Papers (11) | |
| UniProtKB:P57744 · SGD:S000007348 | 15254020 Roesch K, et al. (2004) The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex. Hum Mol Genet 13(18):2101-11 |
| 11956200 Hofmann S, et al. (2002) The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. J Biol Chem 277(26):23287-93 | |
| 11489896 Rothbauer U, et al. (2001) Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem 276(40):37327-34 | |
| 10051608 Koehler CM, et al. (1999) Human deafness dystonia syndrome is a mitochondrial disease. Proc Natl Acad Sci U S A 96(5):2141-6 | |
| UniProtKB:P57744 · SGD:S000007348 | 15254020 Roesch K, et al. (2004) The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex. Hum Mol Genet 13(18):2101-11 |
| 11956200 Hofmann S, et al. (2002) The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space. J Biol Chem 277(26):23287-93 | |
| 11489896 Rothbauer U, et al. (2001) Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem 276(40):37327-34 | |
| 11405338 Bauer MF and Neupert W (2001) Import of proteins into mitochondria: a novel pathomechanism for progressive neurodegeneration. J Inherit Metab Dis 24(2):166-80 | |
| 10611480 Bauer MF, et al. (1999) The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom. FEBS Lett 464(1-2):41-7 | |
| 10051608 Koehler CM, et al. (1999) Human deafness dystonia syndrome is a mitochondrial disease. Proc Natl Acad Sci U S A 96(5):2141-6 | |
| 10051550 Wallace DC and Murdock DG (1999) Mitochondria and dystonia: the movement disorder connection? Proc Natl Acad Sci U S A 96(5):1817-9 | |