| Peroxisomal membrane anchor protein (Pex14p) conserved region | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| Symbol | Pex14_N | ||||||||
| Pfam | PF04695 | ||||||||
| InterPro | IPR006785 | ||||||||
| |||||||||
Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the PEX14 gene. [5] [6]
| Peroxisomal membrane anchor protein (Pex14p) conserved region | |||||||||
|---|---|---|---|---|---|---|---|---|---|
| Identifiers | |||||||||
| Symbol | Pex14_N | ||||||||
| Pfam | PF04695 | ||||||||
| InterPro | IPR006785 | ||||||||
| |||||||||
Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the PEX14 gene. [5] [6]
This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. [6]
PEX14 has been shown to interact with
A peroxisome (IPA:[pɛɜˈɹɒksɪˌsoʊm]) is a membrane-bound organelle, a type of microbody, found in the cytoplasm of virtually all eukaryotic cells. Peroxisomes are oxidative organelles. Frequently, molecular oxygen serves as a co-substrate, from which hydrogen peroxide (H2O2) is then formed. Peroxisomes owe their name to hydrogen peroxide generating and scavenging activities. They perform key roles in lipid metabolism and the reduction of reactive oxygen species.
Protein targeting or protein sorting is the biological mechanism by which proteins are transported to their appropriate destinations within or outside the cell. Proteins can be targeted to the inner space of an organelle, different intracellular membranes, the plasma membrane, or to the exterior of the cell via secretion. Information contained in the protein itself directs this delivery process. Correct sorting is crucial for the cell; errors or dysfunction in sorting have been linked to multiple diseases.
In biochemical protein targeting, a peroxisomal targeting signal (PTS) is a region of the peroxisomal protein that receptors recognize and bind to. It is responsible for specifying that proteins containing this motif are localised to the peroxisome.
Peroxisomal disorders represent a class of medical conditions caused by defects in peroxisome functions. This may be due to defects in single enzymes important for peroxisome function or in peroxins, proteins encoded by PEX genes that are critical for normal peroxisome assembly and biogenesis.
Peroxisomal targeting signal 1 receptor (PTS1R) is a protein that in humans is encoded by the PEX5 gene.
Peroxisome biogenesis factor 1, also known as PEX1, is a protein which in humans is encoded by the PEX1 gene.
Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the PEX19 gene.
ATP-binding cassette sub-family D member 3 is a protein that in humans is encoded by the ABCD3 gene.
Peroxisomal biogenesis factor 2 is a protein that in humans is encoded by the PEX2 gene.
Peroxisome assembly protein 12 is a protein that in humans is encoded by the PEX12 gene.
Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the PEX13 gene. It located on chromosome 2 next to KIAA1841
Peroxisomal biogenesis factor 3 is a protein that in humans is encoded by the PEX3 gene.
Peroxisome assembly factor 2 is a protein that in humans is encoded by the PEX6 gene. PEX6 is an AAA ATPase that localizes to the peroxisome. PEX6 forms a hexamer with PEX1 and is recruited to the membrane by PEX26.
Peroxisome biogenesis factor 10 is a protein that in humans is encoded by the PEX10 gene. Alternative splicing results in two transcript variants encoding different isoforms.
Peroxisomal membrane protein PMP34 is a protein that in humans is encoded by the SLC25A17 gene.
Peroxisomal membrane protein PEX16 is a protein that in humans is encoded by the PEX16 gene.
Peroxisome assembly protein 26 is a protein that in humans is encoded by the PEX26 gene.
Peroxisomal membrane protein 11B is a protein that in humans is encoded by the PEX11B gene. It is involved in the regulation of peroxisome abundance.
Peroxisomal membrane protein 11A is a protein that in humans is encoded by the PEX11A gene.
Mitochondrial fission factor (Mff) is a protein that in humans is encoded by the MFF gene. Its primary role is in controlling the division of mitochondria. Mitochondrial morphology changes by continuous fission in order to create interconnected network of mitochondria. This activity is crucial for normal function of mitochondria. Mff is anchored to the mitochondrial outer membrane through the C-terminal transmembrane domain, extruding the bulk of the N-terminal portion containing two short amino acid repeats in the N-terminal half and a coiled-coil domain just upstream of the transmembrane domain into the cytosol. It has also been shown to regulate peroxisome morphology.