Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds. - Wikidata - wikimedia - TriplyDB

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

http://www.wikidata.org/entity/Q33777739

about

Zellweger spectrum disorders: clinical overview and management approach Emerging novel concept of chaperone therapies for protein misfolding diseases The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder.Identification of Small Molecule Compounds for Pharmacological Chaperone Therapy of Aspartylglucosaminuria Using whole-exome sequencing to identify inherited causes of autism.A founder mutation in the PEX6 gene is responsible for increased incidence of Zellweger syndrome in a French Canadian population Disease specific therapies in leukodystrophies and leukoencephalopathies.Induced pluripotent stem cell models of Zellweger spectrum disorder show impaired peroxisome assembly and cell type-specific lipid abnormalities.Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines Peroxisome biogenesis disorders: Biological, clinical and pathophysiological perspectives.Emerging treatments for pediatric leukodystrophies.A Drosophila model for the Zellweger spectrum of peroxisome biogenesis disorders.The PEX1 ATPase Stabilizes PEX6 and Plays Essential Roles in Peroxisome Biology.Pexophagy is responsible for 65% of cases of peroxisome biogenesis disorders.The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders.Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder.Peroxisome biogenesis disorders.

P2860

Q26775566-3333D59F-2FF1-42DD-ADE8-49969E84CFE9 Q27002907-1922C816-8007-4363-9A0C-6C830FF4990C Q27302267-F81D9DCE-3E91-4FA8-B642-E8D008E94601 Q28820747-9931381B-05E8-4C4B-9D5A-FF5346695868 Q30418126-B540D7C0-A27E-4798-8EB0-ED2DAF3881C0 Q34380791-BF32E3B8-7B53-43DD-B1F8-BC2CDF8FDAEC Q35328494-D7C198F1-B275-43B5-BE34-D972E0550692 Q36005581-40F2F945-F3F2-4D07-BD63-81DE9A140AEE Q37558494-DE68DCD5-DB6D-40D0-B9D2-8A4413301E93 Q38116808-3C3C0F71-1FA8-4E7A-9DCB-6A116611BEE0 Q38506539-52245009-F39F-4BB1-A980-2D80ADB46C85 Q38563090-EA45E465-030F-421E-AD21-7C22B399AEB4 Q38733094-52B76012-4AD5-4D54-BDEB-38960241EA0B Q41867417-FDEDE4DF-C3EF-4525-91F9-2ED48593787E Q42150494-18E8FC94-E0D3-45D8-8409-7C2A2AB5A886 Q42859596-937CCA73-284B-436A-B087-072C61110576 Q55209610-BC6CEE37-D429-4158-9D1A-EA1818070C3B

P2860

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

http://www.wikidata.org/entity/Q33777739

description

2010 nî lūn-bûn

@nan

2010 թուականի Մարտին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի մարտին հրատարակված գիտական հոդված

@hy

2010年の論文

@ja

2010年論文

@yue

2010年論文

@zh-hant

2010年論文

@zh-hk

2010年論文

@zh-mo

2010年論文

@zh-tw

2010年论文

@wuu

name

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@ast

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@en

type

label

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@ast

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@en

prefLabel

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@ast

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@en

P1433

Q33777739-D5BD5E60-340F-4066-B6D3-567562E086B3

P1476

Q33777739-CD1A44DE-8885-4577-B524-202F86A2C8E3

P2093

Q33777739-1B57B88E-58A1-4FDC-B671-B2AD7DA24A6C

Q33777739-4BA80274-0168-4B3C-88F4-52BD1F9EA5E0

Q33777739-679839E1-664A-4C84-B850-380623154472

Q33777739-9BC3964C-2D64-4B70-BF56-4976E0E5263D

Q33777739-C2281D81-087A-4F14-AEBB-41CA9C2A21E4

Q33777739-C7DE92A7-B7B5-4DB2-8F3C-88D31AD97D21

P2860

Q33777739-0F8EE656-58CE-4FB6-B6FF-BE621608F31E

Q33777739-11DEB5D8-20DD-41C7-BA39-D58BD262406B

Q33777739-1211D9EC-6DAC-47D6-A0BC-15F8AB4AF19C

Q33777739-1F383F31-F61A-4510-BAE2-EB4050D761D5

Q33777739-228EA556-BE5E-427B-8C42-A89FCDCFF925

Q33777739-2C745E14-4859-4807-A0A4-8B70FE3BFCA8

Q33777739-2F3FCAD6-FC79-4130-9C7D-9D314BD319B2

Q33777739-31993621-8E05-4550-A58B-F6DE2462EFF2

Q33777739-32E7F289-B947-4DA2-80B5-D046E39B6984

Q33777739-3ECDF9EE-D519-43D1-B5AD-43DC9BA6B224

P304

Q33777739-D803AB6A-ADA4-4D1D-AD1D-5E4BE1CCD613

P31

Q33777739-9864AC9F-0270-422F-BA4F-FCA782848440

P356

Q33777739-7689E060-DDA0-4E53-AA5D-7B2AC712C509

P407

Q33777739-63666B79-8638-40AD-BC3D-35983F27DD68

P433

Q33777739-64C75A35-97BD-4C13-B5E4-14F835DF3D0F

P478

Q33777739-CBFC8A9F-B09A-4236-A362-8582E7A4C5DB

P577

Q33777739-63672176-5EC8-4769-8FFC-70C6C5DDD547

P5875

Q33777739-CBF7EDEB-8785-4A41-B9C5-04C60842C6EB

P698

Q33777739-F2443DE7-F7BA-4B29-BF55-572C675DD076

P932

Q33777739-50E17493-F28F-4858-BB02-0CAE3DB4B1A4

P356

PNAS.0914960107

P1433

Proceedings of the National Academy of Sciences of the United States of America

P1476

Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.

@en

P2093

Ann Snowden

http://www.w3.org/2001/XMLSchema#string

Li Chen

http://www.w3.org/2001/XMLSchema#string

Nancy Braverman

http://www.w3.org/2001/XMLSchema#string

Rui Zhang

http://www.w3.org/2001/XMLSchema#string

Sarn Jiralerspong

http://www.w3.org/2001/XMLSchema#string

Steven Steinberg

http://www.w3.org/2001/XMLSchema#string

P2860

Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p

Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation

A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents

Disruption of a PEX1-PEX6 interaction is the most common cause of the neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease

Functional role of the AAA peroxins in dislocation of the cycling PTS1 receptor back to the cytosol.

Identification of a common PEX1 mutation in Zellweger syndrome

PEX1 mutations in complementation group 1 of Zellweger spectrum patients correlate with severity of disease

Molecular mechanism of a temperature-sensitive phenotype in peroxisomal biogenesis disorder

The bisindolylmaleimide GF 109203X is a potent and selective inhibitor of protein kinase C

AAA+ proteins: have engine, will work

P304

5569-5574

http://www.w3.org/2001/XMLSchema#string

P31

scholarly article

P356

10.1073/PNAS.0914960107

http://www.w3.org/2001/XMLSchema#string

P407

P433

12

http://www.w3.org/2001/XMLSchema#string

P478

107

http://www.w3.org/2001/XMLSchema#string

P577

2010-03-08T00:00:00Z

http://www.w3.org/2001/XMLSchema#dateTime

P5875

41825504

http://www.w3.org/2001/XMLSchema#string

P698

20212125

http://www.w3.org/2001/XMLSchema#string

P932

2851769

http://www.w3.org/2001/XMLSchema#string