Peroxisomal targeting signal 1 receptor (PTS1R) is a

protein Proteins are large biomolecules and macromolecules that comprise one or more long chains of amino acid residues. Proteins perform a vast array of functions within organisms, including catalysing metabolic reactions, DNA replication, respo ...

that in humans is encoded by the ''PEX5''

gene In biology, the word gene (from , ; "...Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a ba ...

. PTS1R is a

peroxisomal A peroxisome () 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 per ...

targeting sequence involved in the specific transport of

molecule A molecule is a group of two or more atoms held together by attractive forces known as chemical bonds; depending on context, the term may or may not include ions which satisfy this criterion. In quantum physics, organic chemistry, and bioch ...

s for

oxidation Redox (reduction–oxidation, , ) is a type of chemical reaction in which the oxidation states of substrate change. Oxidation is the loss of electrons or an increase in the oxidation state, while reduction is the gain of electrons or a d ...

inside the

peroxisome A peroxisome () 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 pero ...

. SKL binds to PTS1R in the

cytosol The cytosol, also known as cytoplasmic matrix or groundplasm, is one of the liquids found inside cells (intracellular fluid (ICF)). It is separated into compartments by membranes. For example, the mitochondrial matrix separates the mitochondri ...

followed by binding to the

Pex14p Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the ''PEX14'' gene. Function This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with i ...

receptor allowing importation of the peroxisomal

through the pexsubunit transporter. Diseases associated with dysfunctional PTS1R receptors include

X-linked Sex linked describes the sex-specific patterns of inheritance and presentation when a gene mutation (allele) is present on a sex chromosome (allosome) rather than a non-sex chromosome (autosome). In humans, these are termed X-linked recessive, ...

adrenoleukodystrophy Adrenoleukodystrophy (ALD) is a disease linked to the X chromosome. It is a result of fatty acid buildup caused by peroxisomal fatty acid beta oxidation which results in the accumulation of very long chain fatty acids in tissues throughout the b ...

and

Zellweger syndrome Zellweger syndrome is a rare congenital disorder characterized by the reduction or absence of functional peroxisomes in the cells of an individual. It is one of a family of disorders called Zellweger spectrum disorders which are leukodystrophie ...

Interactions

PEX5 has been shown to

interact Advocates for Informed Choice, dba interACT or interACT Advocates for Intersex Youth, is a 501(c)(3) nonprofit organization using innovative strategies to advocate for the legal and human rights of children with intersex traits. The organizati ...

with

PEX12 Peroxisome assembly protein 12 is a protein that in humans is encoded by the ''PEX12'' gene. Function PEX12 is needed for protein import into peroxisomes. This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essen ...

PEX13 Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the ''PEX13'' gene. It located on chromosome 2 next to KIAA1841 Interactions PEX13 has been shown to interact with PEX14, PEX5 and PEX19 Peroxisomal biogenesis fa ...

and

PEX14 Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the ''PEX14'' gene. Function This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with i ...

References

External links

GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum

OMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum
{{PDB Gallery, geneid=5830

Interactions

References

Further reading

External links