Peroxisome-Targeting Signal 1 Receptor

A cytoplasmic receptor and peroxin that contains a series of TETRATRICOPEPTIDE REPEATS and binds to PEROXISOME TARGETING SIGNAL 1 (SKL-type). It is essential for protein import into PEROXISOMES; mutations in the PEX5 gene are associated with PEROXISOMAL DISORDERS such as ZELLWEGER SYNDROME.

Also Known As:

PTS1 Receptor; PTS1 Receptors; Peroxin PEX5; Peroxin-5; Peroxisomal Biogenesis Factor 5; Peroxisome Receptor 1; PEX5, Peroxin; Peroxin 5; Peroxisome Targeting Signal 1 Receptor; Receptor, PTS1; Receptors, PTS1

Networked: 7 relevant articles (0 outcomes, 0 trials/studies)

Bio-Agent Context: Research Results

Amino Acids, Peptides, and Proteins: 1
- Proteins: 484843
  - Carrier Proteins: 12999
    - Peroxins: 50
      - Peroxisome-Targeting Signal 1 Receptor: 7
        Arabidopsis PEX5 protein
        human PEX5 protein
        mouse Pex5 protein
        Pichia pastoris PEX5 protein
        rat Pex5 protein
        S cerevisiae PEX5 protein

Experts

1.	Stanley, Will A: 1 article (11/2010)
2.	Williams, Chris P: 1 article (11/2010)
3.	Berg, Jeremy M: 1 article (05/2007)
4.	Maynard, Ernest L: 1 article (05/2007)
5.	Hueni, S E: 1 article (02/2001)
6.	Legakis, J E: 1 article (02/2001)
7.	Subramani, S: 1 article (02/2001)
8.	Terlecky, S R: 1 article (02/2001)

Related Diseases

1.	Peroxisomal Disorders (Peroxisomal Disorder) 08/01/1996 - "The conservation of the PTS1 receptor in several yeasts has led to the cloning and characterization of the gene encoding its human homolog, PTS1R, which is mutated in a group of patients afflicted with fatal peroxisomal disorders. " 02/01/2001 - "No import was detected in fibroblasts from a human patient belonging to complementation group 2, who suffered from the fatal peroxisomal disorder Zellweger syndrome and lacked a functional PTS1 receptor, Pex5p. " 08/01/1996 - "cerevisiae PTS1 receptor resulted in a cytosolic location of chloramphenicol acetyl transferase appended with the human catalase PTS, as did expression of this protein in cells from a neonatal adrenoleukodystrophy patient specifically defective in PTS1 import. "
2.	Zellweger Syndrome (Zellweger's Syndrome) 02/01/2001 - "No import was detected in fibroblasts from a human patient belonging to complementation group 2, who suffered from the fatal peroxisomal disorder Zellweger syndrome and lacked a functional PTS1 receptor, Pex5p. " 05/18/2007 - "Three mutations in the gene encoding Pex5p, the peroxisomal targeting signal type-1 (PTS1) receptor, have been reported, each associated with a disorder of the Zellweger spectrum of different severity. "
3.	Peroxisome biogenesis disorders 02/01/1995 - "Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders." 11/01/2010 - "Peroxin 5 has medical significance in that (i) congenital defects can lead to fatal peroxisome biogenesis disorders, (ii) inefficient peroxisome targeting is linked to disease and aging and (iii) differences between human peroxin 5 and homologues in pathogens may be exploited in the development of therapeutics."
4.	Non-Small-Cell Lung Carcinoma (Carcinoma, Non-Small Cell Lung) 04/01/2023 - "Circular RNA Sec61 subunit alpha isoform 1 by competitive absorption of microRNA-513a-5p mediates peroxisomal biogenesis factor 5 expression and promotes the malignant phenotype of non-small cell lung cancer."
5.	Congenital Abnormalities (Deformity) 11/01/2010 - "Peroxin 5 has medical significance in that (i) congenital defects can lead to fatal peroxisome biogenesis disorders, (ii) inefficient peroxisome targeting is linked to disease and aging and (iii) differences between human peroxin 5 and homologues in pathogens may be exploited in the development of therapeutics."

Related Drugs and Biologics

1.	Protein Isoforms (Isoforms)
2.	Transferases
3.	Proteins (Proteins, Gene)
4.	Chloramphenicol (Chloromycetin)
5.	Catalase
6.	Circular RNA
7.	Peroxisomal Targeting Signals

Related Therapies and Procedures

1.	Therapeutics