نتایج جستجو برای: peroxisome biogenesis

تعداد نتایج: 36041  

Journal: :Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 2016

Journal: :Biochimica et biophysica acta 2006
Ronald J A Wanders Hans R Waterham

Peroxisomal disorders are a group of inherited diseases in man in which either peroxisome biogenesis or one or more peroxisomal functions are impaired. The peroxisomal disorders identified to date are usually classified in two groups including: (1) the disorders of peroxisome biogenesis, and (2) the single peroxisomal enzyme deficiencies. This review is focused on the second group of disorders,...

Journal: :FEBS letters 1995
J A Kiel I K Keizer-Gunnink T Krause M Komori M Veenhuis

PER genes are essential for the biogenesis of peroxisomes in the yeast Hansenula polymorpha. Here we describe the functional complementation of a H. polymorpha per9 disruption strain (delta per9) by a heterologous gene. The Saccharomyces cerevisiae Pas3p, a homologue of per9p, restored peroxisome biogenesis and peroxisomal protein import in the delta per9 mutant, allowing it to grow again on me...

2014
Joseph E. Faust Arvind Manisundaram Pavlina T. Ivanova Stephen B. Milne James B. Summerville H. Alex Brown Michael Wangler Michael Stern James A. McNew

Peroxisomes are ubiquitous organelles that perform lipid and reactive oxygen species metabolism. Defects in peroxisome biogenesis cause peroxisome biogenesis disorders (PBDs). The most severe PBD, Zellweger syndrome, is characterized in part by neuronal dysfunction, craniofacial malformations, and low muscle tone (hypotonia). These devastating diseases lack effective therapies and the developme...

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