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Translocase of Inner Mitochondrial Membrane 8 Homolog A (TIMM8A) takes part in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial. TIMM8A plays a role as a chaperone-like protein which protects the hydrophobic precursors from aggregation and leads them through the mitochondrial intermembrane space. TIMM8A is essential for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Defects in TIMM8A cause Jensen syndrome. TIMM8A and TIMM13, forms a 70 kDa heterohexamer.

40S ribosomal protein S18 (RPS18) is a member of the ribosomal protein S13P family.RPS18 is located in the cytoplasm. Ribosomes, the organelles which catalyze protein synthesis, consist of a small 40S subunit and a large 60S subunit.Jointly these subunits are composed of 4 RNA species and approximately 80 structurally distinct proteins. RPS18 encodes a ribosomal protein which is a component of the 40S subunit. RPS18 is a recognized binding partner of Cofilin and has been proposed to be a novel substrate for CaMKII. Among the diseases associated with RPS18 are bowen-conradi syndrome, and pasteurellosis.

Ribosomes (the organelles that catalyze protein synthesis) consist of a small 40S subunit and a large 60S subunit. Jointly these subunits are composed of 4 RNA species and approximately 80 structurally distinct proteins. RPS10 which is is located in the cytoplasm and a part of the S10E family of ribosomal proteins, is a ribosomal protein that is a component of the 40S subunit. Variable expression of this gene in colorectal cancers compared to adjacent normal tissues has been observed, although no correlation between the level of expression and the severity of the disease has been found.

Translocase of Outer Mitochondrial Membrane 34 (TOMM34) which is found in the cytoplasm and sometimes associated with the outer mitochondrial membrane is involved in the import of precursor proteins into mitochondria. TOMM34 has a chaperone-like activity, binding the mature portion of unfolded proteins and aiding their import into mitochondria. TOMM34 has a weak ATPase activity and contains 6 TPR repeats.

Ribosomal Protein 4X (RPS4X) is ribosomal protein S4, a component of the 40S subunit which is a part from the S4E family of ribosomal proteins. Ribosomal protein S4 is the only ribosomal protein known to be encoded by more than one gene, that is RPS4X and ribosomal protein S4, Y-linked (RPS4Y). Both isoforms encoded by these genes are not the same, but are functionally alike.

Ribosomes, the organelles which catalyze protein synthesis, contain a small 40S subunit and a large 60S subunit. Together these subunits are composed of 4 RNA species and nearly 80 structurally different proteins. RPL34 is a member of the L34E family of ribosomal proteins and it encodes a ribosomal protein which is a component of the 60S subunit. RPL34 is found in the cytoplasm and has been mapped to 4q though it was initially believed it is located at 17q21. Transcript variants which are a result of alternative splicing, alternative transcription initiation sites, and/or alternative polyadenylation exist; all encode the same protein. As is typical for genes encoding ribosomal proteins, there are several processed pseudogenes of this gene spread all over the genome.

RPL23A is a ribosomal protein which is a component of the 60S subunit. Ribosomes, the organelles which catalyze protein synthesis, consist of a small 40S subunit and a large 60S subunit. Together these subunits are composed of 4 RNA species and approximately 80 structurally different proteins. RPL23A is a member of the L23P family of ribosomal proteins. It is located in the cytoplasm. Recombinant Human Ribosomal Protein L23A may be one of the target molecules involved in mediating growth inhibition. The corresponding protein binds to a specific site on the 26S rRNA in yeast.

RTN4IP1 is a unique mitochondrial protein that interacts with reticulon 4, which is an effective inhibitor of regeneration ensuing spinal cord injury. The reticulon 4 interaction with mitochondrial proteins may offer insight into the mechanisms for reticulon-induced inhibition of neurite growth.

SMCP is a protein-coding gene. SMCP localizes to the capsule linked with the mitochondrial outer membranes and assumed to function in the organization and stabilization of the helical structure of the sperm's mitochondrial sheath. Sperm mitochondria vary in morphology and subcellular localization from those of somatic cells. They are elongated, flattened, and arranged circumferentially construct a helical coiled sheath in the midpiece of the sperm flagellum. Diseases associated with SMCP include middle ear disease, and hard palate cancer.

Ribosomal Protein S6 Kinase Beta-1 Isoform A or RPS6KB1 is type of serine/threonine kinas. The protein effects on the PIP3 and phosphoinositide-dependent kinase-1 as part of the PI3 kinase pathway. Protein synthesis is induced by Phosphorylation of S6 in the ribosome. It was shown that phosphorylation of p70S6K at threonine 389 acts as mTOR activation indication and corresponded along with the inhibition of autophagy in different cases, nonetheless new studies has shown that the p70S6K activity has a constructive and increasing affect of autophagy.

Ribosomal Protein S19, also known as RPS19, is a part of the S19E family of ribosomal proteins. RPS19 is located in the cytoplasm and required for pre-rRNA processing and maturation of 40S ribosomal subunits. Mutations in RPS19 trigger Diamond-Blackfan anemia, a constitutional erythroblastopenia characterized by absent or decreased erythroid precursors, in a subset of patients.

DNAJC19 is part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. DNAJC19 is a single-pass membrane protein which contains a J domain and is localized to the inner membrane of the mitochondrion. Expressed ubiquitously, DNAJC19 acts as a component of the mitochondrial DNAJC19 complex that is responsible for the ATP-dependent translocation of select proteins from the inner mitochondrial membrane to the mitochondrial matrix. Defects in the DNAJC19 gene are the cause of 3-methylglutaconic aciduria type 5 (MGA5), otherwise known as dilated cardiomyopathy with ataxia (DCMA).

RPS3A is a member of the S3AE family of ribosomal proteins. Ribosomes, the organelles which catalyze protein synthesis, composed of a large 60S subunit and a small 40S subunit. Together these subunits consist of 4 RNA classes and around 80 structurally diverse proteins. RPS3A, a component of the 40S subunit, is a ribosomal protein that is situated in the cytoplasm. Interference of the gene encoding rat ribosomal protein S3a, in v-fos-transformed rat cells cause a degeneration of the transformed phenotype.

RPS27A is a small protein composed of 76 amino acids. RPS27A is found only in eukaryotic organisms among which shows strong sequence conservation. The RPS27A protein is present in all cell types, thus giving rise to its name.
RPS27A is found either in free form or conjugated to proteins through a covalent bond between the glycine at the C-terminal end and the side chains of lysine.
The connection of multiple copies of RPS27A targets the proteins for degradation by the 26S proteosome. RPS27A ligation is an ATP-dependent multi-step process. RPS27A is activated by the E1 enzyme. The attachment of RPS27A to the target protein is catalyzed by the E2 enzyme acting in concert with E3 which is involved in the recognition of the substrate protein.

Ribosomal protein S2, (RPS2) is a member of the S5P familyof ribosomal proteins. Ribosomes, the organelles which catalyze protein synthesis, consist of a small 40S subunit and a large 60S subunit. Jointly these subunits are composed of 4 RNA species and approximately 80 structurally differentproteins. RPS2 is a ribosomal protein which is a component of the 40S subunit. RPS2located in the cytoplasm. RPS2 shares sequence similarity with mouse LLRep3. RPS2 has been associated with diamond-blackfan anemia, and herpes simplex.

Ribosomal Protein L35, also known as RPL35 is a ribosomal protein which is a component of the 60S subunit. Ribosomes are the organelles which catalyze protein synthesis, they consist of a small 40S subunit and a large 60S subunit. Jointly these subunits are composed of 4 RNA species and about 80 structurally different proteins. RPL35 is a member of the L29P family of ribosomal proteins. RPL35 is located in the cytoplasm. As typicaly for genes encoding ribosomal proteins, there are multiple processed pseudogenes of RPL35 dispersed through the genome.

Cytochrome c oxidase assembly factor 4 (COA4) is the last enzyme of the mitochondrial respiratory chain which needs a great number of accessory factors. COA4 is vital for oxidative phosphorylation and comprises multiple complexes including cytochrome c oxidase, assembled in macromolecular supercomplexes. COX4 is a protein-coding gene which defect in it causes an acute human encephalomyopathies.

Ribosomes, the organelles which catalyze protein synthesis, contain a small 40S subunit and a large 60S subunit. Together these subunits are composed of 4 RNA species and nearly 80 structurally distinct proteins. This gene encodes a ribosomal phosphoprotein which is a component of the 60S subunit. The protein, which is a functional equivalent of the E. coli L7/L12 ribosomal protein, is a member of the L12P family of ribosomal proteins and has a vital part in the elongation step of protein synthesis. In opposed to most ribosomal proteins, which are basic, the encoded protein is acidic.The P1 C-terminal end is nearly identical to the C-terminal ends of the ribosomal phosphoproteins P0 and P2. The P1 protein can interact with P0 and P2 to form a pentameric complex consisting of P1 and P2 dimers, and a P0 monomer. P1 is located in the cytoplasm. As is typical for genes encoding ribosomal proteins, there are multiple processed pseudogenes of this gene distributed all over the genome.

60S ribosomal protein L26-like 1 (RPL26L1) is a member of the ribosomal protein family and has a role in protein synthesis. RPL26L1 is situated in the cytoplasm. RPL26L1 shares a great sequence similarity with ribosomal protein L26. Currently it is unknown whether the RPL26L1 is a functional ribosomal protein or if it has developed a function which is independent of the ribosome.

Translational activator of mitochondrially encoded cytochrome c oxidase I (TACO1) belongs to the TACO1 family. TACO1 is a mitochondrial protein which functions as a translational activator of mitochondrially-encoded cytochrome c oxidase 1. Mutations in this gene are associated with Leigh syndrome.

ATP synthase subunit O (ATP5O) localizes to the mitochondria and catalyzes ATP synthesis. ATP5O is a component of the F-type ATPase found in the mitochondrial matrix. F-type ATPases are composed of a catalytic core and a membrane proton channel. ATP5O seems to be part of the connector connecting these two components and may be involved in transmission of conformational changes or proton conductance.

MECR catalyzes the reduction of trans-2-enoyl-CoA to acyl-CoA with chain length from C6 to C16 in an NADPH dependent manner with preference to medium chain length substrate. MECR protein takes part in the mitochondrial synthesis of fatty acids.

mRNA turnover protein 4 homolog (MRTO4) is a member of the ribosomal protein L10P family. MRTO4 is a protein sharing a low level of sequence similarity with ribosomal protein P0. Though the precise function of the MRTO4 is presently unknown, it seems to be involved in mRNA turnover and ribosome assembly. MRTO4 is a nucleolar component of the ribosome assembly apparatus which shares remarkable similarity and contends for binding to the 25S rRNA GAR domain with the ribosomal protein P0. The MRTO4 gene is located on human chromosome 1, which extends over 260 million base pairs, is comprised of over 3,000 genes and contains approximately 8% of the human genome.

Mmitochondrial import receptor subunit TOMM20 homolog (TOMM20) is a member of the Tom20 family. The Tom machinery consists of import receptors for the initial binding of cytosolically synthesized preproteins and a general import pore (GIP) for the membrane translocation of various preproteins into the mitochondria. TOMM20 acts as the transit peptide receptor at the surface of the mitochondrion outer membrane and facilitates the movement of preproteins into the TOM40 translocation pore.