TIMM9

Last updated
TIMM9
Protein TIMM9 PDB 2bsk.png
Available structures
PDB Ortholog search: PDBe RCSB
Identifiers
Aliases TIMM9 , TIM9, TIM9A, translocase of inner mitochondrial membrane 9
External IDs OMIM: 607384 MGI: 1353436 HomoloGene: 40847 GeneCards: TIMM9
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001024853
NM_001024854
NM_001286203
NM_013896

RefSeq (protein)

NP_001020024
NP_001020025
NP_001273132
NP_038924

Location (UCSC) Chr 14: 58.41 – 58.43 Mb Chr 12: 71.17 – 71.18 Mb
PubMed search [3] [4]
Wikidata
View/Edit Human View/Edit Mouse

Mitochondrial import inner membrane translocase subunit Tim9 is an enzyme that in humans is encoded by the TIMM9 gene. [5] [6] [7]

TIMM9 belongs to a family of evolutionarily conserved proteins that are organized in heterooligomeric complexes in the mitochondrial intermembrane space.

These proteins mediate the import and insertion of hydrophobic membrane proteins into the mitochondrial inner membrane.[supplied by OMIM] [7]

Related Research Articles

<span class="mw-page-title-main">Mitochondrial membrane transport protein</span>

Mitochondrial membrane transport proteins, also known as mitochondrial carrier proteins, are proteins which exist in the membranes of mitochondria. They serve to transport molecules and other factors, such as ions, into or out of the organelles. Mitochondria contain both an inner and outer membrane, separated by the inter-membrane space, or inner boundary membrane. The outer membrane is porous, whereas the inner membrane restricts the movement of all molecules. The two membranes also vary in membrane potential and pH. These factors play a role in the function of mitochondrial membrane transport proteins. There are 53 discovered human mitochondrial membrane transporters, with many others that are known to still need discovered.

<span class="mw-page-title-main">TIMM8A</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit Tim8 A, also known as Deafness-dystonia peptide or protein is an enzyme that in humans is encoded by the TIMM8A gene. This translocase has similarity to yeast mitochondrial proteins that are involved in the import of metabolite transporters from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Deafness-dystonia syndrome and it is postulated that MTS/DFN-1 is a mitochondrial disease caused by a defective mitochondrial protein import system.

<span class="mw-page-title-main">TOMM20</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import receptor subunit TOM20 homolog is a protein that in humans is encoded by the TOMM20 gene. TOM20 is one of the receptor systems of the TOM complex in the outer mitochondrial membrane (OMM).

<span class="mw-page-title-main">TOMM22</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import receptor subunit TOM22 homolog is a protein that in humans is encoded by the TOMM22 gene.

<span class="mw-page-title-main">TIMM13</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit Tim13 is an enzyme that in humans is encoded by the TIMM13 gene.

<span class="mw-page-title-main">TOMM34</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import receptor subunit TOM34 is a protein that in humans is encoded by the TOMM34 gene.

<span class="mw-page-title-main">Translocase of the outer membrane</span>

The translocase of the outer membrane (TOM) is a complex of proteins found in the outer mitochondrial membrane of the mitochondria. It allows movement of proteins through this barrier and into the intermembrane space of the mitochondrion. Most of the proteins needed for mitochondrial function are encoded by the nucleus of the cell. The outer membrane of the mitochondrion is impermeable to large molecules greater than 5000 Daltons. The TOM works in conjunction with the translocase of the inner membrane (TIM) to translocate proteins into the mitochondrion. Many of the proteins in the TOM complex, such as TOMM22, were first identified in Neurospora crassa and Saccharomyces cerevisiae. Many of the genes encoding these proteins are designated as TOMM complex genes.

<span class="mw-page-title-main">TIMM10</span>

Mitochondrial import inner membrane translocase subunit Tim10 is an enzyme that in humans is encoded by the TIMM10 gene.

<span class="mw-page-title-main">MTX1</span> Protein-coding gene in the species Homo sapiens

Metaxin 1, also known as MTX1, is a protein which in humans is encoded by the MTX1 gene.

<span class="mw-page-title-main">TIMM44</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit TIM44 is an enzyme that in humans is encoded by the TIMM44 gene.

<span class="mw-page-title-main">TIMM10B</span>

Mitochondrial import inner membrane translocase subunit Tim9 B is an enzyme that in humans is encoded by the FXC1 gene.

<span class="mw-page-title-main">TOMM70A</span>

Mitochondrial import receptor subunit TOM70 is a protein that in humans is encoded by the TOMM70A gene.

<span class="mw-page-title-main">TIMM23</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit Tim23 is an enzyme that in humans is encoded by the TIMM23 gene.

<span class="mw-page-title-main">TIMM17A</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit Tim17-A is an enzyme that in humans is encoded by the TIMM17A gene.

<span class="mw-page-title-main">TOMM40</span> Protein-coding gene in the species Homo sapiens

Translocase of outer mitochondrial membrane 40 homolog (yeast), also known as TOMM40, is a protein which in humans is encoded by the TOMM40 gene.

<span class="mw-page-title-main">DNAJC19</span>

Mitochondrial import inner membrane translocase subunit TIM14 is an enzyme that in humans is encoded by the DNAJC19 gene on chromosome 3. TIM14 belongs to the DnaJ family, which has been involved in Hsp40/Hsp70 chaperone systems. As a mitochondrial chaperone, TIM14 functions as part of the TIM23 complex import motor to facilitate the import of nuclear-encoded proteins into the mitochondria. TIM14 also complexes with prohibitin complexes to regulate mitochondrial morphogenesis, and has been implicated in dilated cardiomyopathy with ataxia.

<span class="mw-page-title-main">TIMM22</span>

Mitochondrial import inner membrane translocase subunit Tim22 is an enzyme that in humans is encoded by the TIMM22 gene.

<span class="mw-page-title-main">TIMM50</span> Protein-coding gene in the species Homo sapiens

Mitochondrial import inner membrane translocase subunit TIM50 is a protein that in humans is encoded by the TIMM50 gene. Tim50 is a subunit of the Tim23 translocase complex in the inner mitochondrial membrane. Mutations in TIMM50 can lead to epilepsy, severe intellectual disability, and 3-methylglutaconic aciduria. TIMM50 expression is increased in breast cancer cells and decreased in hypertrophic hearts.

The translocase of the inner membrane (TIM) is a complex of proteins found in the inner mitochondrial membrane of the mitochondria. Components of the TIM complex facilitate the translocation of proteins across the inner membrane and into the mitochondrial matrix. They also facilitate the insertion of proteins into the inner mitochondrial membrane, where they must reside in order to function, these mainly include members of the mitochondrial carrier family of proteins.

<span class="mw-page-title-main">ADP/ATP translocase 3</span> Protein-coding gene in the species Homo sapiens

ADP/ATP translocase 3, also known as solute carrier family 25 member 6, is a protein that in humans is encoded by the SLC25A6 gene.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000100575 - Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000021079 - Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Jin H, Kendall E, Freeman TC, Roberts RG, Vetrie DL (Feb 2000). "The human family of Deafness/Dystonia peptide (DDP) related mitochondrial import proteins". Genomics. 61 (3): 259–67. doi:10.1006/geno.1999.5966. PMID   10552927.
  6. Muhlenbein N, Hofmann S, Rothbauer U, Bauer MF (Mar 2004). "Organization and function of the small Tim complexes acting along the import pathway of metabolite carriers into mammalian mitochondria". J Biol Chem. 279 (14): 13540–6. doi: 10.1074/jbc.M312485200 . PMID   14726512.
  7. 1 2 "Entrez Gene: TIMM9 translocase of inner mitochondrial membrane 9 homolog (yeast)".

Further reading