The Peroxisomal PTS1-Import Defect of
ATPases Associated with Diverse Cellular Activities
/ deficiency
Macroautophagy
Membrane Proteins
/ deficiency
Peroxins
/ genetics
Peroxisomal Targeting Signals
/ genetics
Peroxisomes
/ metabolism
Protein Transport
Recombinant Fusion Proteins
/ biosynthesis
Saccharomyces cerevisiae
/ metabolism
Saccharomyces cerevisiae Proteins
/ genetics
Atg36
Pex1
peroxisomal protein import
pexophagy
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
29 Jan 2020
29 Jan 2020
Historique:
received:
10
01
2020
revised:
25
01
2020
accepted:
27
01
2020
entrez:
5
2
2020
pubmed:
6
2
2020
medline:
18
11
2020
Statut:
epublish
Résumé
The important physiologic role of peroxisomes is shown by the occurrence of peroxisomal biogenesis disorders (PBDs) in humans. This spectrum of autosomal recessive metabolic disorders is characterized by defective peroxisome assembly and impaired peroxisomal functions. PBDs are caused by mutations in the peroxisomal biogenesis factors, which are required for the correct compartmentalization of peroxisomal matrix enzymes. Recent work from patient cells that contain the Pex1(G843D) point mutant suggested that the inhibition of the lysosome, and therefore the block of pexophagy, was beneficial for peroxisomal function. The resulting working model proposed that Pex1 may not be essential for matrix protein import at all, but rather for the prevention of pexophagy. Thus, the observed matrix protein import defect would not be caused by a lack of Pex1 activity, but rather by enhanced removal of peroxisomal membranes via pexophagy. In the present study, we can show that the specific block of
Identifiants
pubmed: 32013259
pii: ijms21030867
doi: 10.3390/ijms21030867
pmc: PMC7037794
pii:
doi:
Substances chimiques
Membrane Proteins
0
PEX11 protein, S cerevisiae
0
Peroxins
0
Peroxisomal Targeting Signals
0
Recombinant Fusion Proteins
0
Saccharomyces cerevisiae Proteins
0
ATPases Associated with Diverse Cellular Activities
EC 3.6.4.-
PEX1 protein, S cerevisiae
EC 3.6.4.-
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Deutsche Forschungsgemeinschaft
ID : PerTrans FOR1905
Références
Biochem J. 2004 Nov 15;384(Pt 1):37-45
pubmed: 15283676
Autophagy. 2017 May 4;13(5):991-994
pubmed: 28318378
Autophagy. 2017 May 4;13(5):868-884
pubmed: 28521612
Biochim Biophys Acta Mol Cell Res. 2019 Feb;1866(2):199-213
pubmed: 30408545
Biochim Biophys Acta. 2014 Jan;1843(1):163-81
pubmed: 23545414
Autophagy. 2016;12(1):1-222
pubmed: 26799652
Proc Natl Acad Sci U S A. 1989 Jul;86(14):5419-23
pubmed: 2568633
Cells. 2019 Jun 30;8(7):
pubmed: 31262095
Sci Rep. 2019 Jul 22;9(1):10557
pubmed: 31332264
J Biol Chem. 2006 Sep 15;281(37):27003-15
pubmed: 16849337
Mol Genet Genomic Med. 2017 Jul 06;5(5):531-552
pubmed: 28944237
J Cell Biol. 1999 Mar 22;144(6):1151-62
pubmed: 10087260
Biosci Rep. 2015 May 14;35(3):
pubmed: 26182377
Proc Natl Acad Sci U S A. 2010 Mar 23;107(12):5569-74
pubmed: 20212125
Mol Cell Biol. 2004 Oct;24(20):8895-906
pubmed: 15456864
Am J Hum Genet. 2001 Jul;69(1):35-48
pubmed: 11389485
Annu Rev Biochem. 2006;75:295-332
pubmed: 16756494
Bioessays. 2017 Oct;39(10):
pubmed: 28787099
Biochim Biophys Acta. 2016 May;1863(5):922-33
pubmed: 26611709
J Inherit Metab Dis. 1999 May;22(3):311-3
pubmed: 10384394
Biochim Biophys Acta. 2016 May;1863(5):838-49
pubmed: 26367801
Biochim Biophys Acta. 2012 Sep;1822(9):1430-41
pubmed: 22871920
Autophagy. 2014 May;10(5):835-45
pubmed: 24657987
Am J Hum Genet. 2015 Oct 1;97(4):535-45
pubmed: 26387595
J Cell Biochem. 2019 Mar;120(3):3243-3258
pubmed: 30362618
Proc Natl Acad Sci U S A. 1998 Jul 21;95(15):8630-5
pubmed: 9671729
Biochim Biophys Acta. 2012 Jan;1823(1):150-8
pubmed: 21963882
Cell Cycle. 2017;16(21):2037-2045
pubmed: 28933989
EMBO J. 2012 Jun 29;31(13):2852-68
pubmed: 22643220
Nat Rev Mol Cell Biol. 2013 Dec;14(12):803-17
pubmed: 24263361
Mol Cells. 2018 Jan 31;41(1):55-64
pubmed: 29370694
Yeast. 1998 Jan 30;14(2):115-32
pubmed: 9483801