Protein biogenesis. Medical search. Frequent questions

Ribosome biogenesis protein BRX1 homolog (BRIX1) also known as brix domain-containing protein 2 (BXDC2) is a protein that in humans is encoded by the BRIX1 gene. (wikipedia.org)
The RNA-binding protein Hfq is important for ribosome biogenesis and affects translation fidelity. (ox.ac.uk)
Ribosome biogenesis is a complex process involving multiple factors. (ox.ac.uk)
Our work expands the functions of the Sm-like protein Hfq beyond its function in small RNA-mediated regulation and unveils a novel role of Hfq as crucial in ribosome biogenesis and translation. (ox.ac.uk)
Ribosome biogenesis in eukaryotes requires the participation of a large number of ribosome assembly factors. (rcsb.org)
The highly conserved eukaryotic nucleolar protein Nep1 has an essential but unknown function in 18S rRNA processing and ribosome biogenesis. (rcsb.org)
This suggests the participation of Nep1 in a methyltransferase reaction during ribosome biogenesis. (rcsb.org)

An infantile form of Refsum disease also exists and is an autosomal recessive disorder of peroxisomal biogenesis, leading to many biochemical abnormalities, including elevated plasma concentration of phytanic acid, pristanic acid, very long chain fatty acids, and C27 bile acids. (medscape.com)
This defect of peroxisomal biogenesis is caused by PEX7 gene mutations and characterized by skeletal changes that include midface hypoplasia, strikingly short proximal limbs, frontal bossing, small nares, cataracts, ichthyosis, and profound psychomotor retardation. (msdmanuals.com)

RD can be classified as a peroxisome biogenesis disorder. (medscape.com)
Refsum disease can be classified as a peroxisome biogenesis disorder. (medscape.com)
[ 1 ] Infantile Refsum disease is a peroxisome biogenesis disorder. (medscape.com)

The endoplasmic reticulum (ER) membrane protein complex (EMC) has been shown to be a key factor in the biosynthesis and stable expression of multi-pass transmembrane proteins, and its loss is thought to cause retinal degeneration. (bioquicknews.com)

Transcriptome analyses, real-time PCR, and immunoblotting showed consistent reductions in the expression of the large and small ribosomal protein subunits (RPL and RPS, respectively) in hyperammonemic murine skeletal myotubes, HEK cells, and skeletal muscle from hyperammonemic rats and human cirrhotics. (nih.gov)
This gene encodes a ribosomal protein that is a component of the 40S subunit. (nih.gov)
Human ribosomal protein S16 inhibites excision of the first intron from its own]. (nih.gov)
Binding of human ribosomal protein S16 with the 18S rRNA fragment 1203-1236/1521-1698]. (nih.gov)
Here we use biochemical and cryoelectron microscopy analyses to show that the amino-terminal amphipathic helix of SecA and the ribosomal protein uL23 form a composite binding site for the transmembrane domain (TMD) on the nascent protein. (nih.gov)
Also, protein-protein interaction network analysis revealed that cytochrome c oxidase and 60S ribosomal protein played key roles to detoxify the multimetal. (ufz.de)
In addition, yeast Nep1 binds to a 6-nt RNA-binding motif also found in 18S rRNA and facilitates the incorporation of ribosomal protein Rps19 during the formation of pre-ribosomes. (rcsb.org)

Organelle Biogenesis and Protein Translocation. (hhmi.org)

In both kingdoms of life, however, the same membrane that acquaints the newly born membrane protein also synthesizes the bilayer lipids and thus ensures the balanced growth of the membrane as a whole. (unifr.ch)
While understanding this interdependence between bilayer lipids and protein biogenesis is interesting in its own right, careful consideration of a potential host's membrane lipid composition may also allow optimization of the yield and activity of membrane proteins that are expressed in a heterologous organism. (unifr.ch)
Here, we review and discuss some examples that illustrate the interdependence between bilayer lipids and the biogenesis of integral membrane proteins. (unifr.ch)

Saal KA, Richter F, Rehling P, Rizzoli SO (2018) Combined Use of Unnatural Amino Acids Enables Dual-Color Super-Resolution Imaging of Proteins via Click Chemistry. (uni-goettingen.de)

Among several newly identified proteins, we focused on a highly conserved but uncharacterized protein (VIPP1-Associated protein 1, VIA1) that robustly interacts with VIPP1 in both systems. (biorxiv.org)
we found that components of the inner membrane subcomplex consisting of PilMNOP were not essential for surface pilus expression, whereas the highly conserved inner membrane protein PilC was essential. (mcmaster.ca)
Structural organization of essential iron-sulfur clusters in the evolutionarily highly conserved ATP-binding cassette protein ABCE1. (nature.com)

Several genetic knockout (KO) screens identified the ER membrane protein complex (EMC) as a critical host component for flavivirus infection. (biorxiv.org)

Similar to cellular TM proteins, the viral polyprotein relies on the host cell machinery for targeting to the ER, translocation across the membrane and insertion of transmembrane domains (TMDs) into the lipid bilayer during its translation. (biorxiv.org)
For most TM proteins, recognition of a hydrophobic TMD or signal sequence by the SRP followed by transfer to the Sec61 translocation channel are sufficient for membrane targeting and accurate topogenesis ( Shao and Hegde, 2011 ). (biorxiv.org)
Recent evidence indicates that the lipid composition of the host membrane can determine the fate of the newborn membrane protein, as it can affect (1) the efficiency of translocation, (2) the topology of the resulting membrane protein, (3) its stability, (4) its assembly into oligomeric complexes, (5) its transport and sorting along the secretory pathway, and (6) its enzymatic activity. (unifr.ch)

Cells need ribosomes in order to create proteins. (europa.eu)
Ribosomes consist of ribonucleic acids (RNAs) and proteins. (europa.eu)
Ribosomes, the organelles that catalyze protein synthesis, consist of a small 40S subunit and a large 60S subunit. (nih.gov)
The essential ATP-binding cassette protein ABCE1 splits 80S ribosomes into 60S and 40S subunits after canonical termination or quality-control-based mRNA surveillance processes. (nature.com)

DRB2 has been remarkably conserved throughout plant evolution, raising the possibility that translational repression is the ancient form of miRNA-directed gene regulation in plants, and that Dicer partnering proteins, such as human TRBP, might play a similar role in other eukaryotic systems. (nature.com)
Figure 4: Translational and post-translational regulation of DRB1 and DCL1, and evolutionary conservation of DRB1 and DRB2 proteins. (nature.com)
Depletion of UbKEKS induces accumulation of lamin A in the nucleoli, highlighting the need for deeper investigations about protein composition and functions regulation of this highly dynamic and membrane-less compartment. (bvsalud.org)

The overall process of mitochondrial protein biogenesis consists of four stages: (1) protein synthesis, (2) targeting the protein to the mitochondria, (3) transporting the protein into the mitochondrial lumen, and (4) targeting the protein to its final destination in the organelle. (reasons.org)
Each stage of mitochondrial protein biogenesis involves multiple steps with each one carried out by an ensemble of proteins. (reasons.org)
If not, the entire process of mitochondrial protein biogenesis fails. (reasons.org)

The TJ consists of transmembrane proteins occludin, claudins, and junctional adhesion molecules (JAMs), which are organized in intramembranous strands and are linked to the F-actin cytoskeleton either directly or indirectly through members of the MAGUK (membrane-associated guanylate kinase) family of proteins ZO-1, -2, and -3. (rupress.org)

Protein import into this mitochondrial sub-compartment is underpinned by an intriguing variety of pathways, many of which are still poorly understood. (uni-koeln.de)
We discuss the main import pathways into the IMS, but also how IMS proteins are degraded or even retro-translocated to the cytosol in an integrated network of interactions that is necessary to maintain a healthy balance of IMS proteins under physiological and cellular stress conditions. (uni-koeln.de)
We conclude this review by highlighting new and exciting perspectives in this area with a view to develop a better understanding of yet unknown, likely unconventional import pathways, how presequence-less proteins can be targeted and the basis for dual localisation in the IMS and the cytosol. (uni-koeln.de)
MiRNA-378a-3p acts as a negative regulator of mitochondrial metabolic and biogenesis pathways. (cdc.gov)

How do post-transcriptional events modulate the expression of functional macromolecules, including proteins and non-coding RNAs? (nih.gov)

ALMS1 encodes a ~ 0.5 megadalton protein that localises to the base of centrioles. (springer.com)
SMN1 encodes the SMN protein, which is part of a multiprotein complex required for the biogenesis of small nuclear ribonucleoproteins. (medscape.com)
It encodes a protein of 38.6 kd. (medscape.com)

One of the few known critical regulators of this process, VIPP1 (Vesicle-Inducing Protein in Plastids 1), was recently shown to be structurally similar to ESCRT-III proteins - the first evidence for ESCRT-like (Endosomal Sorting Complex Required for Transport) machinery in chloroplasts. (biorxiv.org)
Together these subunits are composed of 4 RNA species and approximately 80 structurally distinct proteins. (nih.gov)
Fe-S clusters can act as redox sensors or catalysts and are found to be used by a large number of functional and structurally diverse proteins. (hal.science)

Lastly, we showed a physical interaction between the EMC and DENV NS4B protein post-cleavage and rapid degradation of processed NS4B in the absence of EMC. (biorxiv.org)

Of the few known proteins involved in these processes, VIPP1 stands out due to its similarity to ESCRT-III, an integral component of the ESCRT machinery that is responsible for membrane remodeling and trafficking in the cytoplasm of eukaryotes. (biorxiv.org)
UBIQUITIN-like modifier proteins that function in CYTOPLASM to VACUOLE transport (Cvt), vesicle transport within the GOLGI APPARATUS, and AUTOPHAGOSOME biogenesis. (bvsalud.org)

PEX1, also known as Peroxisome biogenesis factor 1, is a 1,283 amino acid protein that is 143 kDa, cytoplasmic but is often anchored to a peroxisomal membrane where it forms a heteromeric complex and plays a role in the import of proteins into peroxisomes and peroxisome biogenesis. (novusbio.com)
Direct insertion of PMPs into peroxisomes depends on Pex3 and Pex19 8 , Pex19 being the chaperone/receptor for PMPs and Pex3 the peroxisomal docking protein for the Pex19-cargo complex. (rug.nl)

SMN1 has been linked to pre-mRNA splicing, spliceosome biogenesis, and the nucleolar protein fibrillarin. (medscape.com)
Using data-independent acquisition mass spectrometry and microscopy, we show that despite not impacting protein stability, UbKEKS is required to maintain a normal nucleolar organization. (bvsalud.org)

Insertion of proteins into the bacterial outer membrane is mediated by a dedicated chaperone machinery. (unibas.ch)

Over the past decades, new ubiquitin variants and ubiquitin-like proteins arose to further enrich this mechanism. (bvsalud.org)

Receptor proteins that are part of the TOM complex recognize the signal sequence and transport the protein through a channel within the TOM interior into the intermembrane space (the region between the mitochondrian's inner and outer membranes). (reasons.org)
These processes are facilitated by the signal recognition particle (SRP), the translocon-associated protein (TRAP) complex (also known as signal-sequence receptor complex), the Sec61 translocon and the signal peptidase complex. (biorxiv.org)
MybiosourceProduct Short Name: [G-Protein Coupled Receptor 126 (GPR126)]Product Name Synonyme: [G-Protein Coupled Receptor. (biocheminfo.org)

In their absence, the bulk of the matrix proteins are mislocalized to the cytosol 5 , even though these cells still contain peroxisomal membrane structures to which peroxisomal membrane proteins (PMPs) are sorted 6 . (rug.nl)

Except for select proteins, most mitochondrial proteins are made in the cytosol of the cell and transported into the mitochondria. (reasons.org)

TOM (translocase of the outer membrane) and TIM (translocase of the inner membrane) function as biomolecular machines that operate in tandem to import newly made proteins into mitochondria. (reasons.org)
Finally, if the protein is to be incorporated into the outer membrane, then another complex referred to as SAM inserts it into the outer membrane. (reasons.org)
At least eight different chaperones, arranged in an assembly line, transport β-barrel outer membrane proteins (Omps) and fold them into the membrane. (unibas.ch)
Figure 2: Outer membrane biogenesis by BAM. (unibas.ch)

In this process, TOM and TIM aren't brothers, but large protein complexes found in the outer and inner membranes of mitochondria. (reasons.org)
Once in the intermembrane space (the region between the outer and inner membranes), two different TIM complexes (TIM22 and TIM23) work together, taking the protein "baton" from the TOM complex and ushering the protein into the lumen (or the matrix) of the mitochondria. (reasons.org)
The biogenesis and maintenance of thylakoid membranes is a critical yet underappreciated area of research. (biorxiv.org)
Significance Statement Thylakoid membranes are essential for photosynthesis, yet their biogenesis and maintenance are poorly understood. (biorxiv.org)
The endoplasmic reticulum (ER) is a network of membranes that folds, modifies, and transports proteins in eukaryotic cells. (cshlpress.com)

How Complex Is Protein Transport into Mitochondria? (reasons.org)
If the protein is to be incorporated into the inner membrane, it possesses an additional targeting sequence that is recognized by another protein complex dubbed OXA. (reasons.org)
If the protein is to carry out its work in the intermembrane space, then the OXA complex will transport the protein back across the inner membrane. (reasons.org)
The team has previously shown that the protein Rab1 is involved in the transport of materials from the endoplasmic reticulum to Golgi, that glycosylphoshatidylinositol (GPI) synthesis is necessary for rhodopsin sorting in the trans-Golgi network, and that the Rab11/dRip11/Myo V complex is essential for post-Golgi transport of rhodopsin. (bioquicknews.com)
In line with a more complex picture, multiple isoforms of the protein likely exist and non-centrosomal sites of localisation have been reported. (springer.com)
Tiam1 interacts with Par3 and aPKCζ, which are two components of the conserved Par3-Par6-aPKC polarity complex, and triggers biogenesis of the TJ through the activation of Rac and aPKCζ, which is independent of Cdc42. (rupress.org)
Our data indicate that Tiam1-mediated activation of Rac in PAs controls TJ biogenesis and polarity in epithelial cells by association with and activation of the Par3-Par6-aPKC polarity complex. (rupress.org)
[ 15 ] The surface of the APC contains 2 peptide-binding proteins (ie, major histocompatability complex [MHC] classes I and II), which can stimulate cytotoxic T (T C ) cells, regulatory T (Treg) cells, and helper T (T H ) cells. (medscape.com)

The EMC facilitates accurate insertion, topology and/or stabilization of specific cellular TM proteins including a subset of tail-anchored proteins and G protein-coupled receptors. (biorxiv.org)

Overexpression of GSK3β-resistant variants, genetic depletion of IκB kinase β (IKKβ) (activated during hyperammonemia), protein interactions, and in vitro kinase assays showed that IKKβ phosphorylated β-catenin directly. (nih.gov)
Structure of the human protein kinase MPSK1 reveals an atypical activation loop architecture. (nih.gov)

Decreased ribosomal content was accompanied by decreased expression of cMYC, a positive regulator of ribosomal biogenesis, as well as reduced expression and activity of β-catenin, a transcriptional activator of cMYC. (nih.gov)
MicroRNA-7641 is a regulator of ribosomal proteins and a promising targeting factor to improve the efficacy of cancer therapy. (nih.gov)

Proteins dubbed chaperones keep the mitochondrial proteins unfolded and stabilized throughout this process. (reasons.org)
Molecular chaperones play a key role in cellular processes, including protein homeostasis, but also in membrane protein transport and biogenesis. (unibas.ch)
Recently, we have systematically investigated how molecular chaperones interact with the intrinsically disordered protein α-synuclein. (unibas.ch)

We show that hyperammonemia, which occurs in diverse chronic disorders, impairs protein synthesis as a result of decreased ribosomal content and translational capacity. (nih.gov)
Our data show that decreased protein synthesis during hyperammonemia is mediated via a novel GSK3β-independent, IKKβ-dependent impairment of the β-catenin-cMYC axis. (nih.gov)

Argonaute proteins exhibit small RNA-guided RNA endonuclease activity, often referred to as Slicer activity. (elsevierpure.com)
Animal germline cells express PIWI subclade proteins of the Argonaute superfamily. (elsevierpure.com)

This second project involved the protein Maf1, which inhibited Pol III activity in yeast and mammalian cells. (europa.eu)

In Escherichia coli, the essential ATPase SecA was found to cotranslationally target a subset of nascent membrane proteins to the SecYEG translocase at the plasma membrane. (nih.gov)

Together, our results suggest that the EMC engages with DENV polyproteins to ensure proper biogenesis of the NS4A-NS4B region, and provide further evidence for the cellular function of the EMC in the stable expression of TM proteins. (biorxiv.org)
They are responsible for the ubiquitination of â ¼20% of cellular proteins degraded through the proteasome, by catalyzing the transfer of E2-loaded ubiquitin to a substrate. (bvsalud.org)
Compared with individual exposures, co-exposure aerosols produced greater acellular and cellular oxidants detected by electron paramagnetic resonance (EPR) spectroscopy, and in vivo immune-spin trapping (IST), as well as synergistically increased lavage neutrophils, lavage proteins and inflammation related gene/protein expression. (cdc.gov)

Flaviviruses such as dengue (DENV), Zika (ZIKV) or West Nile virus (WNV) translate their genome as a single multi-pass transmembrane (TM) protein at the endoplasmic reticulum (ER) membrane. (biorxiv.org)
Peroxisomal membrane proteins insert into the endoplasmic reticulum. (rug.nl)

English Most integral membrane proteins are cotranslationally inserted into the lipid bilayer. (unifr.ch)
These EV contain a lipid bilayer associated to membrane proteins. (frontiersin.org)

We find the ssDNA-binding complexes canonical replication protein A (RPA1, RPA2, and RPA3) and Alternative-RPA (RPA1, RPA3, and primate-specific RPA4) are upregulated in Huntington disease and spinocerebellar ataxia type 1 (SCA1) patient brains. (bvsalud.org)

In this study, we report a new role for the Krüppel-type ZNF-containing protein ZNF432 as a novel poly(ADP-ribose) (PAR) reader that regulates the DNA damage response. (bvsalud.org)

Several versions of the SMN protein are produced by SMN2, but only one version (isoform d) is complete and functional. (medscape.com)
[ 14 ] Thus, only 10-15% of all functional SMN protein is produced from SMN2 . (medscape.com)
Recently discovered ubiquitin variant UbKEKS can specifically target several proteins and yet, functional consequences of this new modification remain unknown. (bvsalud.org)

Here, we cover current knowledge of the SUF multiprotein machinery that synthesizes and inserts Fe-S clusters into proteins. (hal.science)

The mitochondrial intermembrane space (IMS) houses a large spectrum of proteins with distinct and -critical functions. (uni-koeln.de)
How do distinct transcriptional regulatory proteins coordinate their job with extrinsic factors and the microenvironment? (nih.gov)

Here we show that the form of regulatory action directed by a plant miRNA is determined by DRB2, a DICER-LIKE1 (DCL1) partnering protein. (nature.com)
Furthermore, our results reveal that the core silencing proteins ARGONAUTE1 (AGO1) and SERRATE (SE) are highly regulated by miRNA-guided translational inhibition. (nature.com)
Figure 2: Accumulation of miRNA target mRNAs and proteins in the shoot apex of drb1 and drb2 . (nature.com)
MiRNA-378a regulated proteins involved in mitochondrial fusion, transcription, and fatty acid metabolism. (cdc.gov)

Taken together, our results support the emerging concept that ZNF-containing proteins can modulate PARylation, which can be embodied by the pivotal role of ZNF432 to finely balance the outcome of PARPi response by regulating homologous recombination. (bvsalud.org)

Instead, absence of EMC led to a large fraction of expressed viral proteins being targeted to the proteasome post-translationally. (biorxiv.org)

Even though biochemists are figuring out how the process of protein transport works, they must also identify a plausible evolutionary pathway that can adequately account for the evolution of this biochemical operation. (reasons.org)

Anatomy56

Organisms13

Diseases4

Chemicals and Drugs94

Analytical, Diagnostic and Therapeutic Techniques and Equipment20

Phenomena and Processes65

Information Science4

Ribosome biogenesis7

Peroxisomal biogenesis2

Peroxisome biogenesis disorder3

Biosynthesis1

Ribosomal protein7

Organelle Biogenesis1

Lipids3

Amino Acids1

Highly conserved3

Membrane protein complex1

Translocation3

Ribosomes4

Regulation3

Mitochondrial protein3

Transmembrane proteins1

Pathways4

RNAs1

Encodes3

Structurally3

Degradation1

Cytoplasm2

Peroxisomes2

Nucleolar2

Bacterial1

Mechanism1

Receptor3

Matrix proteins1

Cytosol1

Outer-membrane4

Membranes5

Complex8

Insertion1

Kinase2

Regulator2

Chaperones3

Synthesis2

ARGONAUTE2

Yeast1

Subset1

Cellular3

Endoplasmic2

Lipid bilayer2

Complexes1

Regulates1

Functional3

Multiprotein1

Distinct2

MiRNA4

Modulate1

Proteasome1

Biochemical1