A missense allele of PEX5 is responsible for the defective import of PTS2 cargo proteins into peroxisomes

M Ali, SY Khan, TA Rodrigues, T Francisco, X Jiao… - Human genetics, 2021 - Springer
… of peroxisome targeting signal 1 (PTS1) cargo protein, be monoubiquitinated and exported
back into the cytosol. Importantly, the mutant … a novel missense mutation in PEX5 responsible …

[HTML][HTML] The type-2 peroxisomal targeting signal

M Kunze - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2020 - Elsevier
… to the second peroxisomal targeting signal (PTS1) is unclear. … biogenesis disorders, PBDs)
due to mutations in genes … as a cross-complementation between the mutation HS3E in the …

Peroxisome: metabolic functions and biogenesis

K Okumoto, S Tamura, M Honsho, Y Fujiki - … Models, Peroxisomal …, 2020 - Springer
… is encoded by a single gene and imported into peroxisomes by the PTS1 receptor Pex5 in a
… by genetic phenotype-complementation assay with a peroxisome-defective CHO cell mutant

[HTML][HTML] Laboratory diagnosis of disorders of peroxisomal biogenesis and function: a technical standard of the American College of Medical Genetics and Genomics …

I De Biase, S Tortorelli, L Kratz, SJ Steinberg… - Genetics in …, 2020 - Elsevier
… and implementing clinical biochemical genetic testing for peroxisomal disorders, …
Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome

[HTML][HTML] Insights into the structure and function of the Pex1/Pex6 AAA-ATPase in peroxisome homeostasis

RM Judy, CJ Sheedy, BM Gardner - Cells, 2022 - mdpi.com
… -terminal peroxisome-targeting signal (PTS1) that is definedMutations in the PEX genes
that affect peroxisome formation or the import of peroxisome matrix proteins cause peroxisome

Neonatal adrenoleukodystrophy/disorders of peroxisomal biogenesis

WL Nyhan, GF Hoffmann, AI Al-Aqeel… - Atlas of Inherited …, 2020 - taylorfrancis.com
… the same group of disorders of peroxisomal biogenesis, but mutations in at least 13 PEX …
Mutations in the PTS1 receptor gene PXR1 define complementation group 2 of the peroxisome

[HTML][HTML] PEX5 translocation into and out of peroxisomes drives matrix protein import

ML Skowyra, TA Rapoport - Molecular cell, 2022 - cell.com
… shuttles between cytosol and peroxisomes and releases cargo … receptor and cargo release.
Our results reveal the unique mechanism by which PEX5 ferries proteins into peroxisomes

[HTML][HTML] The peroxisomal matrix shuttling receptor Pex5 plays a role in FB1 production and virulence in Fusarium verticillioides

WY Yu, LIN Mei, H Yan, JJ Wang, SM Zhang… - Journal of Integrative …, 2022 - Elsevier
… Here, we report that PTS1 shuttling receptor FvPex5 is involved in … deletion, we generated
a gene-complementation strain Δ… We suggest that the ΔFvpex5 mutant in F. verticillioides has …

Laboratory diagnosis of disorders of peroxisomal biogenesis andfunction: a technical standard of the American College of Medical Genetics and Genomics (ACMG)

S Tortorelli, L Kratz, JS Steven, CO Kristina… - Genetics in …, 2020 - search.proquest.com
Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome
… PEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders…

[PDF][PDF] Insights into the Structure and Function of the Pex1/Pex6 AAA-ATPase in Peroxisome Homeostasis. Cells 2022, 11, 2067

RM Judy, CJ Sheedy, BM Gardner - 2022 - pdfs.semanticscholar.org
… Most matrix proteins use a C-terminal peroxisometargeting signal (PTS1) that is defined by
… a suppressor mutant in PEX1 that restored peroxisome function in a PEX6-defective mutant, …