208 related articles for article (PubMed ID: 30362618)
1. Zellweger spectrum disorder patient-derived fibroblasts with the PEX1-Gly843Asp allele recover peroxisome functions in response to flavonoids.
MacLean GE; Argyriou C; Di Pietro E; Sun X; Birjandian S; Saberian P; Hacia JG; Braverman NE
J Cell Biochem; 2019 Mar; 120(3):3243-3258. PubMed ID: 30362618
[TBL] [Abstract][Full Text] [Related]
2. Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.
Zhang R; Chen L; Jiralerspong S; Snowden A; Steinberg S; Braverman N
Proc Natl Acad Sci U S A; 2010 Mar; 107(12):5569-74. PubMed ID: 20212125
[TBL] [Abstract][Full Text] [Related]
3. The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder.
Hiebler S; Masuda T; Hacia JG; Moser AB; Faust PL; Liu A; Chowdhury N; Huang N; Lauer A; Bennett J; Watkins PA; Zack DJ; Braverman NE; Raymond GV; Steinberg SJ
Mol Genet Metab; 2014 Apr; 111(4):522-532. PubMed ID: 24503136
[TBL] [Abstract][Full Text] [Related]
4. A
Gonzalez KL; Ratzel SE; Burks KH; Danan CH; Wages JM; Zolman BK; Bartel B
Proc Natl Acad Sci U S A; 2018 Apr; 115(14):E3163-E3172. PubMed ID: 29555730
[TBL] [Abstract][Full Text] [Related]
5. Liver disease predominates in a mouse model for mild human Zellweger spectrum disorder.
Berendse K; Boek M; Gijbels M; Van der Wel NN; Klouwer FC; van den Bergh-Weerman MA; Shinde AB; Ofman R; Poll-The BT; Houten SM; Baes M; Wanders RJA; Waterham HR
Biochim Biophys Acta Mol Basis Dis; 2019 Oct; 1865(10):2774-2787. PubMed ID: 31207289
[TBL] [Abstract][Full Text] [Related]
6. Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities.
Wang XM; Yik WY; Zhang P; Lu W; Huang N; Kim BR; Shibata D; Zitting M; Chow RH; Moser AB; Steinberg SJ; Hacia JG
Stem Cell Res Ther; 2015 Aug; 6():158. PubMed ID: 26319495
[TBL] [Abstract][Full Text] [Related]
7. Novel PEX1 mutations in fibroblasts from children with Zellweger spectrum disorders exhibit temperature sensitive characteristics.
Liang JS; Hung KL; Lin LJ; Ong WP; Keng WT; Lu JF
Epilepsy Behav; 2023 Aug; 145():109266. PubMed ID: 37385119
[TBL] [Abstract][Full Text] [Related]
8. Disorders of peroxisome biogenesis due to mutations in PEX1: phenotypes and PEX1 protein levels.
Walter C; Gootjes J; Mooijer PA; Portsteffen H; Klein C; Waterham HR; Barth PG; Epplen JT; Kunau WH; Wanders RJ; Dodt G
Am J Hum Genet; 2001 Jul; 69(1):35-48. PubMed ID: 11389485
[TBL] [Abstract][Full Text] [Related]
9. A common PEX1 frameshift mutation in patients with disorders of peroxisome biogenesis correlates with the severe Zellweger syndrome phenotype.
Maxwell MA; Nelson PV; Chin SJ; Paton BC; Carey WF; Crane DI
Hum Genet; 1999; 105(1-2):38-44. PubMed ID: 10480353
[TBL] [Abstract][Full Text] [Related]
10. The Peroxisomal PTS1-Import Defect of
Mastalski T; Brinkmeier R; Platta HW
Int J Mol Sci; 2020 Jan; 21(3):. PubMed ID: 32013259
[TBL] [Abstract][Full Text] [Related]
11. Accurate and live peroxisome biogenesis evaluation achieved by lentiviral expression of a green fluorescent protein fused to a peroxisome targeting signal 1.
Demaret T; Courtoy GE; Ravau J; Van Der Smissen P; Najimi M; Sokal EM
Histochem Cell Biol; 2020 May; 153(5):295-306. PubMed ID: 32124009
[TBL] [Abstract][Full Text] [Related]
12. A longitudinal study of retinopathy in the PEX1-Gly844Asp mouse model for mild Zellweger Spectrum Disorder.
Argyriou C; Polosa A; Cecyre B; Hsieh M; Di Pietro E; Cui W; Bouchard JF; Lachapelle P; Braverman N
Exp Eye Res; 2019 Sep; 186():107713. PubMed ID: 31254513
[TBL] [Abstract][Full Text] [Related]
13. Longitudinal study of Pex1-G844D NMRI mouse model: A robust pre-clinical model for mild Zellweger spectrum disorder.
Demaret T; Roumain M; Ambroise J; Evraerts J; Ravau J; Bouzin C; Bearzatto B; Gala JL; Stepman H; Marie S; Vincent MF; Muccioli GG; Najimi M; Sokal EM
Biochim Biophys Acta Mol Basis Dis; 2020 Nov; 1866(11):165900. PubMed ID: 32693164
[TBL] [Abstract][Full Text] [Related]
14. The PEX1 ATPase Stabilizes PEX6 and Plays Essential Roles in Peroxisome Biology.
Rinaldi MA; Fleming WA; Gonzalez KL; Park J; Ventura MJ; Patel AB; Bartel B
Plant Physiol; 2017 Aug; 174(4):2231-2247. PubMed ID: 28600347
[TBL] [Abstract][Full Text] [Related]
15. Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder.
Berendse K; Ebberink MS; Ijlst L; Poll-The BT; Wanders RJ; Waterham HR
Orphanet J Rare Dis; 2013 Sep; 8():138. PubMed ID: 24016303
[TBL] [Abstract][Full Text] [Related]
16. Depletion of HNRNPA1 induces peroxisomal autophagy by regulating PEX1 expression.
Park NY; Jo DS; Park SJ; Lee H; Bae JE; Hong Y; Kim JB; Kim YH; Park HJ; Choi JY; Lee HJ; Ryoo ZY; Lee HS; Kim JC; Lee EK; Cho DH
Biochem Biophys Res Commun; 2021 Mar; 545():69-74. PubMed ID: 33545634
[TBL] [Abstract][Full Text] [Related]
17. Insights into the Structure and Function of the Pex1/Pex6 AAA-ATPase in Peroxisome Homeostasis.
Judy RM; Sheedy CJ; Gardner BM
Cells; 2022 Jun; 11(13):. PubMed ID: 35805150
[TBL] [Abstract][Full Text] [Related]
18. Structural Mapping of Missense Mutations in the Pex1/Pex6 Complex.
Schieferdecker A; Wendler P
Int J Mol Sci; 2019 Aug; 20(15):. PubMed ID: 31374812
[TBL] [Abstract][Full Text] [Related]
19. Disparate peroxisome-related defects in Arabidopsis pex6 and pex26 mutants link peroxisomal retrotranslocation and oil body utilization.
Gonzalez KL; Fleming WA; Kao YT; Wright ZJ; Venkova SV; Ventura MJ; Bartel B
Plant J; 2017 Oct; 92(1):110-128. PubMed ID: 28742939
[TBL] [Abstract][Full Text] [Related]
20. Novel PEX1 mutations and genotype-phenotype correlations in Australasian peroxisome biogenesis disorder patients.
Maxwell MA; Allen T; Solly PB; Svingen T; Paton BC; Crane DI
Hum Mutat; 2002 Nov; 20(5):342-51. PubMed ID: 12402331
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]