✦ LIBER ✦

Pharmacological induction of peroxisomes in peroxisome biogenesis disorders

✍ Scribed by Heming Wei; Stephan Kemp; Martina C. McGuinness; Ann B. Moser; Kirby D. Smith

Publisher: John Wiley and Sons
Year: 2000
Tongue: English
Weight: 727 KB
Volume: 47
Category: Article
ISSN: 0364-5134
DOI: 10.1002/1531-8249(200003)47:3<286::aid-ana3>3.0.co;2-b

No coin nor oath required. For personal study only.

📜 SIMILAR VOLUMES

Biogenesis of peroxisomes in mammals

✍ Priv. Doz. Dr Wilhelm W. Just; Ubaldo Soto 📂 Article 📅 1992 🏛 John Wiley and Sons 🌐 English ⚖ 644 KB

In this review we would like to summarize our current knowledge on the biogenesis of mammalian peroxisomes and discuss in detail single steps which led to the current model of peroxisome biogenesis. Since some recent results obviously are not fully compatible with this model a revised version is dis

α-Synuclein abnormalities in mouse model

α-Synuclein abnormalities in mouse models of peroxisome biogenesis disorders

✍ Eugenia Yakunin; Ann Moser; Virginie Loeb; Ann Saada; Phyllis Faust; Denis I. Cr 📂 Article 📅 2009 🏛 John Wiley and Sons 🌐 English ⚖ 330 KB

## Abstract α‐Synuclein (αS) is a presynaptic protein implicated in Parkinson's disease (PD). Growing evidence implicates mitochondrial dysfunction, oxidative stress, and αS–lipid interactions in the gradual accumulation of αS in pathogenic forms and its deposition in Lewy bodies, the pathological

Phenotype-genotype relationships in PEX1

Phenotype-genotype relationships in PEX10-deficient peroxisome biogenesis disorder patients

✍ Daniel S. Warren; Brian D. Wolfe; Stephen J. Gould 📂 Article 📅 2000 🏛 John Wiley and Sons 🌐 English ⚖ 453 KB

The peroxisome biogenesis disorders (PBD) are characterized by neural, hepatic, and renal deficiencies, severe mental retardation, and are often lethal. These disorders are genetically and phenotypically heterogeneous and are caused by defective peroxisomal protein import and decreased peroxisomal m

Peroxisomal disorders in man

✍ Professor; Dr Adelbert A. Roscher; Boris Rolinski 📂 Article 📅 1992 🏛 John Wiley and Sons 🌐 English ⚖ 640 KB

PEX1 mutations in the Zellweger spectrum

PEX1 mutations in the Zellweger spectrum of the peroxisome biogenesis disorders

✍ Denis I. Crane; Megan A. Maxwell; Barbara C. Paton 📂 Article 📅 2005 🏛 John Wiley and Sons 🌐 English ⚖ 211 KB

Diseases of the Zellweger spectrum represent a major subgroup of the peroxisome biogenesis disorders, a group of autosomal-recessive diseases that are characterized by widespread tissue pathology, including neurodegeneration. The Zellweger spectrum represents a clinical continuum, with Zellweger syn

Rapid diagnosis of peroxisome biogenesis

Rapid diagnosis of peroxisome biogenesis disorders through immunofluorescence staining of buccal smears

✍ Zhongyi Zhang; Yasuyuki Suzuki; Nobuyuki Shimozawa; Naomi Kondo 📂 Article 📅 2000 🏛 John Wiley and Sons 🌐 English ⚖ 542 KB

Truncating mutations in CCM1, encoding KRIT1, cause hereditary cavernous angiomas, 1 and 65% of non-Hispanic American families have been linked to CCM1 with no founder effects. 2 The common mutation, 742C3 T transition, recently described in 16 of 21 families, 3 supports the previously described str