Deletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain

scientific article published on 4 October 2011

Deletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain is …
instance of (P31):
scholarly articleQ13442814

External links are
P356DOI10.1242/DMM.007708
P932PMC publication ID3255551
P698PubMed publication ID21954064

P50authorEveline Baumgart-VogtQ47158610
P2093author name stringBarbara Ahlemeyer
Magdalena Gottwald
P2860cites workExpression of PEX11beta mediates peroxisome proliferation in the absence of extracellular stimuliQ22004167
Cystic Fibrosis Heterozygote Resistance to Cholera Toxin in the Cystic Fibrosis Mouse ModelQ22299398
Phenotype-genotype relationships in peroxisome biogenesis disorders of PEX1-defective complementation group 1 are defined by Pex1p-Pex6p interactionQ24291411
Fis1, DLP1, and Pex11p coordinately regulate peroxisome morphogenesisQ24301650
PEX11alpha is required for peroxisome proliferation in response to 4-phenylbutyrate but is dispensable for peroxisome proliferator-activated receptor alpha-mediated peroxisome proliferationQ24537850
A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parentsQ24613849
Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group IQ24645143
Disruption of a PEX1-PEX6 interaction is the most common cause of the neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum diseaseQ24681184
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye bindingQ25938984
A new mathematical model for relative quantification in real-time RT-PCRQ27860781
Pex11p plays a primary role in medium-chain fatty acid oxidation, a process that affects peroxisome number and size in Saccharomyces cerevisiaeQ27934293
Peroxisome biogenesis disordersQ28207254
Adrenoleukodystrophy protein-deficient mice represent abnormality of very long chain fatty acid metabolismQ28236420
The PEX Gene Screen: molecular diagnosis of peroxisome biogenesis disorders in the Zellweger syndrome spectrumQ28293113
Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)Q28360949
An immunocytochemical analysis of the ontogeny of the microtubule-associated proteins MAP-2 and Tau in the nervous system of the rat.Q42040001
Influence of heterozygosity for parkin mutation on onset age in familial Parkinson disease: the GenePD studyQ42688205
Temperature sensitive acyl-CoA oxidase import in group A peroxisome biogenesis disordersQ43075476
Retinoic acid reduces apoptosis and oxidative stress by preservation of SOD protein levelQ43615227
Optimized protocols for the simultaneous preparation of primary neuronal cultures of the neocortex, hippocampus and cerebellum from individual newborn (P0.5) C57Bl/6J miceQ46638273
Adreno-leukodystrophy: oxidative stress of mice and men.Q46829099
The sequential appearance of low- and high-molecular-weight forms of MAP2 in the developing cerebellum.Q47176242
Axonal loss and neuroinflammation caused by peroxisome-deficient oligodendrocytesQ48099707
The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidationQ48636627
Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathyQ48671536
Lack of adrenoleukodystrophy protein enhances oligodendrocyte disturbance and microglia activation in mice with combined Abcd1/Mag deficiencyQ50276649
Developmental regulation of microtubule-associated protein 2 expression in regions of mouse brain.Q52061599
Developmental regulation of MAP2 variants during neuronal differentiation in vitro.Q52204718
Differential expression of peroxisomal matrix and membrane proteins during postnatal development of mouse brain.Q53531489
Clinical Spectrum of Homozygous and Heterozygous PINK1 Mutations in a Large German Family With Parkinson DiseaseQ60332322
Motor system abnormalities in heterozygous relatives of a D90A homozygous CuZn-SOD ALS patient of finnish extractionQ73128582
Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger miceQ80060299
Pex11pbeta-mediated growth and division of mammalian peroxisomes follows a maturation pathwayQ84582760
Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalitiesQ28508098
Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotypeQ28511106
Differential expression of rat brain synaptic proteins in development and agingQ28572694
A mouse model for Zellweger syndromeQ28590797
Abnormal cerebellar histogenesis in PEX2 Zellweger mice reflects multiple neuronal defects induced by peroxisome deficiencyQ28591457
Impaired neuronal migration and endochondral ossification in Pex7 knockout mice: a model for rhizomelic chondrodysplasia punctataQ28591869
Detection of peroxisomal proteins and their mRNAs in serial sections of fetal and newborn mouse organsQ28593347
PEX11β induces peroxisomal gene expression and alters peroxisome number during early Xenopus laevis developmentQ31007869
Oxidative metabolism, apoptosis and perinatal brain injury.Q31904904
Targeted inactivation of the X-linked adrenoleukodystrophy gene in miceQ32159154
Visualizing the distribution of synapses from individual neurons in the mouse brainQ33633904
Heterozygous carriers of a Parkin or PINK1 mutation share a common functional endophenotypeQ33652645
Human red blood cell polymorphisms and malariaQ33997329
PEX11 beta deficiency is lethal and impairs neuronal migration but does not abrogate peroxisome functionQ34280995
PEX13 deficiency in mouse brain as a model of Zellweger syndrome: abnormal cerebellum formation, reactive gliosis and oxidative stressQ34458791
X-linked adrenoleukodystrophy: clinical, biochemical and pathogenetic aspects.Q34562917
The evolution of gene duplicatesQ34586458
Cellular and molecular aspects of Zellweger syndrome and other peroxisome biogenesis disordersQ34777961
Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophyQ34811374
Reactive oxygen species and peroxisomes: struggling for balanceQ34982318
Mitochondrial mechanisms of cell death and neuroprotection in pediatric ischemic and traumatic brain injuryQ34988707
A review of morphological techniques for detection of peroxisomal (and mitochondrial) proteins and their corresponding mRNAs during ontogenesis in mice: application to the PEX5-knockout mouse with Zellweger syndromeQ35125097
Is there a phenotype/genotype correlation in peroxisome biogenesis disorders (PBDs)?Q35624394
Peroxisome biogenesis disorders: the role of peroxisomes and metabolic dysfunction in developing brainQ36114812
Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorderQ36274978
A mouse model for X-linked adrenoleukodystrophyQ36566796
Survival advantage of the hemochromatosis C282Y mutationQ37056091
Gene expression phenotype in heterozygous carriers of ataxia telangiectasiaQ37202992
Getting a camel through the eye of a needle: the import of folded proteins by peroxisomesQ37690350
Peroxisomes as dynamic organelles: peroxisomal matrix protein importQ37772547
Superoxide production in rat hippocampal neurons: selective imaging with hydroethidineQ39428776
PMP70 knock-down generates oxidative stress and pro-inflammatory cytokine production in C6 glial cellsQ39920011
Evidence for increased oxidative stress in peroxisomal D-bifunctional protein deficiencyQ40639063
Peroxisomal disorders: genotype, phenotype, major neuropathologic lesions, and pathogenesis.Q41698061
Disorders of peroxisome biogenesis due to mutations in PEX1: phenotypes and PEX1 protein levelsQ42036408
P433issue1
P407language of work or nameEnglishQ1860
P304page(s)125-140
P577publication date2011-10-04
P1433published inDisease Models & MechanismsQ1524006
P1476titleDeletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain
P478volume5

Reverse relations

cites work (P2860)
Q34047964A New Immunomodulatory Role for Peroxisomes in Macrophages Activated by the TLR4 Ligand Lipopolysaccharide
Q35549268Compromised peroxisomes in idiopathic pulmonary fibrosis, a vicious cycle inducing a higher fibrotic response via TGF-β signaling.
Q30840454Coupling organelle inheritance with mitosis to balance growth and differentiation
Q35110393Gene expression patterns in the hippocampus during the development and aging of Glud1 (Glutamate Dehydrogenase 1) transgenic and wild type mice
Q35601225Genome-Wide Localization Study of Yeast Pex11 Identifies Peroxisome-Mitochondria Interactions through the ERMES Complex.
Q64092469High-resolution atmospheric-pressure MALDI mass spectrometry imaging workflow for lipidomic analysis of late fetal mouse lungs
Q90145796Impaired Peroxisomal Fitness in Obese Mice, a Vicious Cycle Exacerbating Adipocyte Dysfunction via Oxidative Stress
Q57071055Infection-Induced Peroxisome Biogenesis Is a Metabolic Strategy for Herpesvirus Replication
Q39062984Knockdown of Pex11β reveals its pivotal role in regulating peroxisomal genes, numbers, and ROS levels in Xenopus laevis A6 cells
Q33778861MicroRNAs upregulated during HIV infection target peroxisome biogenesis factors: Implications for virus biology, disease mechanisms and neuropathology
Q38956337Microporation is an efficient method for siRNA-induced knockdown of PEX5 in HepG2 cells: evaluation of the transfection efficiency, the PEX5 mRNA and protein levels and induction of peroxisomal deficiency
Q51146919Peroxisomal membrane channel Pxmp2 in the mammary fat pad is essential for stromal lipid homeostasis and for development of mammary gland epithelium in mice.
Q92528793Peroxisomes in Immune Response and Inflammation
Q34095390Potential targets for protecting against hippocampal cell apoptosis after transient cerebral ischemia-reperfusion injury in aged rats
Q90008081The HIV-1 Accessory Protein Vpu Downregulates Peroxisome Biogenesis
Q27302267The Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder
Q36632900The gene expression profiles of induced pluripotent stem cells from individuals with childhood cerebral adrenoleukodystrophy are consistent with proposed mechanisms of pathogenesis

Search more.