Results 81 to 90 of about 1,135,071 (192)

Newly identified Chinese hamster ovary cell mutants are defective in biogenesis of peroxisomal membrane vesicles (Peroxisomal ghosts), representing a novel complementation group in mammals

, 1998
We isolated peroxisome biogenesis-defective mutants from Chinese hamster ovary cells by the 9-(1'-pyrene)nonanol/ultraviolet (P9OH/UV) method. Seven cell mutants, ZP116, ZP119, ZP160, ZP161, ZP162, ZP164, and ZP165, of 11 P9OH/UV-resistant cell clones ...
Ghaedi, K., Suzuki, Y., Kinoshita, N., Matsuzono, Y., Shimozawa, N., Kondo, N., Fujiki, Y., Imanaka, T., Wanders, R. J., Okumoto, K.   +9 more
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The peroxisomal membrane protein Pex3p of Hansenula polymorpha: analysis of its function in peroxisome biogenesis [PDF]

, 1999
In this thesis the methytotrophic yeast Hansenula polymorpha was used as a model organism to study peroxisome biogenesis. H. polymorpha cells are able to grow on methanol as a sole carbon source, which requires the compartmentalization of part of its ...
Baerends, Richard Jan Steven,, Baerends, Richard Jan Steven   +1 more
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Peroxisomes and peroxisomal disorders: The main facts

, 2010
International audienceThe importance of peroxisomes for human health is highlighted by the number of peroxisomal disorders (PDs), diseases associated to peroxisome biogenesis disorders and peroxisomal enzyme/transporter deficiencies.
Fidaleo, Marco, Fidaleo M.
core   +1 more source

Cellular mechanisms of peroxisomal biogenesis and maintenance

Peroxisomes are single membrane-bound organelles essential for cellular lipid metabolism, detoxification of reactive oxygen species, and maintenance of metabolic homeostasis.
Fatima, Kaneez
core  

Peroxisomal biogenesis disorders: A review about the molecular background, classification and recent developments

, 2012
Peroxisomes are organelles found in virtually all eukaryotic organisms, fulfilling a variety of universal and also very species-specific tasks. In humans, peroxisomes accomplish essential functions, such as β-oxidation of very long chain fatty acids ...
Degener, F.
core  

Peroxisome Biogenesis Disorder [PDF]

, 2020
openaire   +3 more sources

A new type of peroxisomal disorder with variable expression in liver and fibroblasts.

, 1994
We describe two siblings, presently 5 and 9 years of age, who had neurodegenerative symptoms after the first year of life. Although they lacked clinical characteristics of a peroxisomal disorder, they had elevated levels of plasma very long chain fatty ...
Roels, Frank, MANDEL, H, LUDER, A, WANDERS, RJA, IANCU, TC, AIZIN, A, SCHUTGENS, RBH, Sofer, N., Espeel, Marc, SOFER, N, BERANT, M, Berant, M., Schutgens, R. B., Iancu, T. C., Luder, A., Aizin, A., Roels, F., Espeel, M., Mandel, H., Wanders, R. J.   +19 more
core   +1 more source

Genotype–Phenotype Correlations in Disorders of Peroxisome Biogenesis

Molecular Genetics and Metabolism, 1999
Genetically determined human peroxisomal disorders are subdivided into two major categories: disorders of peroxisome biogenesis (PBD), in which the organelle is not formed normally, and those that involve a single peroxisomal enzyme. Twelve PBD have been identified, and the molecular defects have been defined in 10. All involve defects in the import of
openaire   +2 more sources

Peroxisome biogenesis disorders

Trends in Genetics, 2000
Stephen J Gould, David Valle
openaire   +1 more source

Reply: Low bone mineral density is a common feature of Zellweger spectrum disorders

Molecular Genetics and Metabolism Reports, 2016
Eric T. Rush, Jennifer L. Goodwin, Nancy E. Braverman, William B. Rizzo   +3 more
doaj   +1 more source