What are PEX genes?

PEX genes encode proteins called peroxins and are involved in either peroxisome formation, peroxisomal protein import, or both. As a consequence, mutations in PEX genes cause a deficiency of functional peroxisomes.

What is PEX protein?

PEX genes encode proteins involved in peroxisome biogenesis and proliferation. The molecular processes in peroxisome biogenesis in different organisms were put into context, showing that peroxisomes are not static organelles in eukaryotic evolution.

What is the function of PEX5 in peroxisomes?

The peroxisomal import receptor PEX5 functions as a stress sensor, retaining catalase in the cytosol in times of oxidative stress.

What protein binds newly made peroxisome membrane proteins Pmps in the cytoplasm?

Pex19p
Pex19p binds and stabilizes newly synthesized class 1 mPTS-containing peroxisomal membrane proteins (PMP) in the cytoplasm, and transports them to peroxisomes via anchoring to Pex3p.

What happens to peroxisomes in Zellweger syndrome?

If the production of peroxisomes is altered, these structures cannot perform their usual functions. The signs and symptoms of severe Zellweger spectrum disorder are due to the absence of functional peroxisomes within cells.

What is peroxisome biogenesis disorder?

The peroxisome biogenesis disorders (PBD) are a heterogeneous group of autosomal recessive disorders in which peroxisome assembly is impaired, leading to multiple peroxisome enzyme deficiencies, complex developmental sequelae and progressive disabilities.

What does PEX5 associate with?

peroxisomal matrix proteins
PEX5 is a cytosolic receptor that binds newly translated peroxisomal matrix proteins (cargo) through their peroxisome targeting sequence 1 (PTS1) [14].

Which proteins mediate division fission of both mitochondria and peroxisomes?

Both mitochondrial and peroxisomal fission is facilitated by dynamin-related protein 1 (Drp1)6,7,8,9,10, a member of the dynamin superfamily of proteins that self-assemble as helical scaffolds and utilizes the energy from GTP hydrolysis to constrict and remodel tubular membrane intermediates11,12.

What is peroxisome disorder?

Peroxisomal disorders are a heterogeneous group of inborn errors of metabolism that result in impairment of peroxisome function. In most cases, this results in neurologic dysfunction of varying extent.

How is PEX3 delivered to the peroxisome?

PEX16 and PEX3 are delivered to the ER and are delivered to peroxisomes via vesicular traffic. This route has been characterised in mammalian cells [20]. PEX19 is required for PEX3 exit from the ER in S. cerevisiae (which lacks PEX16).

What is the function of PEX3 in PEX19?

“PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins.” Cited for: FUNCTION, MUTAGENESIS OF LEU-125 AND ASN-134. “The cytosolic domain of PEX3, a protein involved in the biogenesis of peroxisomes, binds membrane lipids.”

What is the structure of the cytosolic domain of PEX3?

The crystal structure of the cytosolic domain of PEX3 in complex with a PEX19-derived peptide. PEX3 adopts a novel fold that is best described as a large helical bundle. The cytosolic domain of PEX3, a protein involved in the biogenesis of peroxisomes, binds membrane lipids.

Does Arabidopsis PEX19 bind to multiple peroxisomes?

Arabidopsis PMP22 inserts directly into peroxisomes in vitro [51] and in vivo [52] and Arabidopsis PEX19 binds multiple PMPs [22]. ii. Direct insertion of PEX3 in a PEX19 and PEX16 dependent manner. This pathway has been described in mammalian cells [41] but has not been examined in any plant system. iii.