Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder. - Wikidata - awgldk - TriplyDB

Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder.

Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder.

http://www.wikidata.org/entity/Q36274978

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Pathogenesis of peroxisomal deficiency disorders (Zellweger syndrome) may be mediated by misregulation of the GABAergic system via the diazepam binding inhibitor Peroxisomes in brain development and function Peroxisomes are required for lipid metabolism and muscle function in Drosophila melanogaster Cerebellum development and medulloblastoma Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype Defects in myelination, paranode organization and Purkinje cell innervation in the ether lipid-deficient mouse cerebellum Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model Docosahexaenoic acid deficit is not a major pathogenic factor in peroxisome-deficient mice Genomic organization, expression analysis, and chromosomal localization of the mouse PEX3 gene encoding a peroxisomal assembly protein PEX2 is the E3 ubiquitin ligase required for pexophagy during starvation.alpha-Synuclein abnormalities in mouse models of peroxisome biogenesis disorders.Peroxisome biogenesis and peroxisome biogenesis disorders.Drosophila carrying pex3 or pex16 mutations are models of Zellweger syndrome that reflect its symptoms associated with the absence of peroxisomes Mechanisms and disturbances of neuronal migration.PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome function Peripheral nervous system defects in a mouse model for peroxisomal biogenesis disorders.Genetic and molecular bases of peroxisome biogenesis disorders.PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stress.Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.Transcriptional evidence for the role of chronic venlafaxine treatment in neurotrophic signaling and neuroplasticity including also Glutamatergic [corrected] - and insulin-mediated neuronal processes.Molecular control of neuronal migration.Peroxisomes in dental tissues of the mouse.Genetic-dependency of peroxisomal cell functions - emerging aspects.Deletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain Peroxisomes in Different Skeletal Cell Types during Intramembranous and Endochondral Ossification and Their Regulation during Osteoblast Differentiation by Distinct Peroxisome Proliferator-Activated Receptors.Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice Metabolic and molecular aspects of ethanolamine phospholipid biosynthesis: the role of CTP:phosphoethanolamine cytidylyltransferase (Pcyt2).Peroxisome deficient invertebrate and vertebrate animal models Peroxisome biogenesis and human peroxisome-deficiency disorders.Peroxisome deficiency causes a complex phenotype because of hepatic SREBP/Insig dysregulation associated with endoplasmic reticulum stress.LRP1 regulates peroxisome biogenesis and cholesterol homeostasis in oligodendrocytes and is required for proper CNS myelin development and repair.The mouse gene encoding the peroxisomal membrane protein 1-like protein (PXMP1-L): cDNA cloning, genomic organization and comparative expression studies.The peroxisomal import proteins PEX2, PEX5 and PEX7 are differently involved in Podospora anserina sexual cycle.From peroxisomal disorders to common neurodegenerative diseases - the role of ether phospholipids in the nervous system.The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation.

P2860

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P2860

Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder.

http://www.wikidata.org/entity/Q36274978

description

1997 nî lūn-bûn

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Targeted deletion of the PEX2 ...... n neuronal migration disorder.

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Targeted deletion of the PEX2 ...... n neuronal migration disorder.

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P2860

A human gene responsible for Zellweger syndrome that affects peroxisome assembly

Miller-Dieker lissencephaly gene encodes a subunit of brain platelet-activating factor acetylhydrolase [corrected]

A unified nomenclature for peroxisome biogenesis factors

Single-Step Method of RNA Isolation by Acid Guanidinium Thiocyanate–Phenol–Chloroform Extraction

Mode of cell migration to the superficial layers of fetal monkey neocortex

Isolation of a Miller-Dieker lissencephaly gene containing G protein beta-subunit-like repeats

Lissencephaly and other malformations of cortical development: 1995 update

Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene

A nonmammalian homolog of the PAF1 gene (Zellweger syndrome) discovered as a gene involved in caryogamy in the fungus Podospora anserina

Restoration by a 35K membrane protein of peroxisome assembly in a peroxisome-deficient mammalian cell mutant

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