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PMID:19479899

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Citation

Toro, AA, Araya, CA, Córdova, GJ, Arredondo, CA, Cárdenas, HG, Moreno, RE, Venegas, A, Koenig, CS, Cancino, J, Gonzalez, A and Santos, MJ (2009) Pex3p-dependent peroxisomal biogenesis initiates in the endoplasmic reticulum of human fibroblasts. J. Cell. Biochem. 107:1083-96

Abstract

The mechanisms of peroxisomal biogenesis remain incompletely understood, specially regarding the role of the endoplasmic reticulum (ER) in human cells, where genetic disorders of peroxisome biogenesis lead to Zellweger syndrome (ZS). The Pex3p peroxisomal membrane protein (PMP) required for early steps of peroxisome biogenesis has been detected in the ER in yeast but not in mammalian cells. Here, we show that Pex3p-GFP expressed in a new ZS cell line (MR), which lacks peroxisomes due to a mutation in the PEX3 gene, localizes first in the ER and subsequently in newly formed peroxisomes. Pex3p bearing an artificial N-glycosylation site shows an electrophoretic shift indicative of ER targeting while en route to preformed peroxisomes in normal fibroblast. A signal peptide that forces its entry into the ER does not eliminate its capability to drive peroxisome biogenesis in ZS cells. Thus, Pex3p is able to drive peroxisome biogenesis from the ER and its ER pathway is not privative of ZS cells. Cross-expression experiments of Pex3p in GM623 cells lacking Pex16p or Pex16p in MR cells lacking Pex3p, showed evidence that Pex3p requires Pex16p for ER location but is dispensable for the ER location of Pex16p. These results indicate that Pex3p follows the ER-to-peroxisomal route in mammalian cells and provides new clues to understand its function.

Links

PubMed Online version:10.1002/jcb.22210

Keywords

Acyltransferases; Case-Control Studies; Catalase; Endoplasmic Reticulum/enzymology; Endoplasmic Reticulum/metabolism; Fibroblasts/cytology; Humans; Lipoproteins/genetics; Lipoproteins/metabolism; Lipoproteins/physiology; Membrane Proteins/genetics; Membrane Proteins/metabolism; Membrane Proteins/physiology; Mutation; Peroxisomes/metabolism; Protein Transport; Zellweger Syndrome

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:CATA

located_in

GO:0005777: peroxisome

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000057)

Seeded From UniProt

complete

HUMAN:THIK

located_in

GO:0005777: peroxisome

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:ABCD3

located_in

GO:0005777: peroxisome

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0002620)

Seeded From UniProt

complete

HUMAN:PEX3

located_in

GO:0005783: endoplasmic reticulum

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:PEX3

involved_in

GO:0007031: peroxisome organization

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:PEX16

located_in

GO:0005783: endoplasmic reticulum

ECO:0000314: direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

HUMAN:PEX16

involved_in

GO:0007031: peroxisome organization

ECO:0000315: mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

HUMAN:PEX16

involved_in

GO:0106101: ER-dependent peroxisome localization

ECO:0000314: direct assay evidence used in manual assertion

P

  • has_input:(UniProtKB:P56589)

Seeded From UniProt

complete

HUMAN:CATA

part_of

GO:0005777: peroxisome

ECO:0000314: direct assay evidence used in manual assertion

C

part_of:(CL:0000057)

Seeded From UniProt

complete

See also

References

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