α-Synuclein abnormalities in mouse models of peroxisome biogenesis disorders
نویسندگان
چکیده
منابع مشابه
Mouse Models with Peroxisome Biogenesis Defects
Peroxisomes are ubiquitous organelles in mammalian cells but it is still unclear how they contribute to normal development and tissue homeostasis. To address this question, gene targeting techniques have been applied on several peroxins to interfere with peroxisome biogenesis in mice. Both peroxins involved in peroxisomal matrix import and peroxins necessary for peroxisome division were inactiv...
متن کاملPeroxisome biogenesis disorders
Defects in PEX genes impair peroxisome assembly and multiple metabolic pathways confined to this organelle, thus providing the biochemical and molecular bases of the peroxisome biogenesis disorders (PBD). PBD are divided into two types--Zellweger syndrome spectrum (ZSS) and rhizomelic chondrodysplasia punctata (RCDP). Biochemical studies performed in blood and urine are used to screen for the P...
متن کاملPeroxisome biogenesis and human peroxisome-deficiency disorders
Peroxisome is a single-membrane-bounded ubiquitous organelle containing a hundred different enzymes that catalyze various metabolic pathways such as β-oxidation of very long-chain fatty acids and synthesis of plasmalogens. To investigate peroxisome biogenesis and human peroxisome biogenesis disorders (PBDs) including Zellweger syndrome, more than a dozen different complementation groups of Chin...
متن کاملdiagnosis of human peroxisomal biogenesis disorders: molecular understanding of peroxisome
peroxisomes are single membrane bound organelles present in a wide variety of eukaryotes from yeast to human, have different functions, two of which are well conserved, i.e. hydrogen peroxide decomposition and fatty acid beta-oxidation. the process of peroxisome biogenesis can be divided into distinct steps including peroxisome membrane assembly, import of matrix proteins and peroxisome prolife...
متن کاملEvolving models for peroxisome biogenesis☆
Significant progress has been made towards our understanding of the mechanism of peroxisome formation, in particular concerning sorting of peroxisomal membrane proteins, matrix protein import and organelle multiplication. Here we evaluate the progress made in recent years. We focus mainly on progress made in yeasts. We indicate the gaps in our knowledge and discuss conflicting models.
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ژورنال
عنوان ژورنال: Journal of Neuroscience Research
سال: 2009
ISSN: 0360-4012,1097-4547
DOI: 10.1002/jnr.22246