Results 31 to 40 of about 1,135,071 (192)

A role for Vps1p, actin, and the Myo2p motor in peroxisome abundance and inheritance in Saccharomyces cerevisiae [PDF]

, 2001
In vivo time-lapse microscopy reveals that the number of peroxisomes in Saccharomyces cerevisiae cells is fairly constant and that a subset of the organelles are targeted and segregated to the bud in a highly ordered, vectorial process.
Marlene van den Berg, Hettema, E.H., Peter Philippsen, Henk F. Tabak, Hoepfner, D., Tabak, H.F., Dominic Hoepfner, Tabak, H. F., Hettema, E. H., Ewald H. Hettema, van den Berg, M., Philippsen, P.   +11 more
core   +2 more sources

Long-Term Cholic Acid Treatment in a Patient with Zellweger Spectrum Disorder

Case Reports in Gastroenterology, 2018
Zellweger spectrum disorders (ZSDs) are a subgroup of peroxisomal biogenesis disorders with a generalized defect in peroxisome function. Liver disease in ZSDs has been associated with the lack of peroxisomal β-oxidation of C27-bile acid intermediates to ...
James E. Heubi, Warren P. Bishop
doaj   +1 more source

Molecular basis of peroxisomal biogenesis disorders caused by defects in peroxisomal matrix protein import [PDF]

, 2012
Peroxisomal biogenesis disorders (PBDs) represent a spectrum of autosomal recessive metabolic disorders that are collectively characterized by abnormal peroxisome assembly and impaired peroxisomal function. The importance of this ubiquitous organelle for
Nagotu, Shirisha, Kalel, Vishal C., Erdmann, Ralf, Platta, Harald W.   +3 more
core   +1 more source

Investigating the Role of Peroxisomal Biogenesis Factor 16 in Pancreatic β-cells [PDF]

, 2023
Peroxisome biogenesis is a complex process that involves the formation of peroxisomal membranes and import of peroxisomal proteins. Peroxisomal biogenesis factor 16 (Pex16) is a critical factor for the recruitment of membrane proteins for the formation ...
Oh, Soo Jung
core   +1 more source

Hansenula polymorpha: An attractive model organism for molecular studies of peroxisome biogenesis and function [PDF]

, 1992
In wild-type Hansenula polymorpha the proliferation of peroxisomes is induced by various unconventional carbon- and nitrogen sources. Highest induction levels, up to 80% of the cytoplasmic volume, are observed in cells grown in methanol-limited chemostat
Titorenko, V.,, Titorenko, V., Klei, I.J. van der; id_orcid, Klei, I.J. van der,, Veenhuis, M., Harder, W.,, Klei, I.J. van der, Veenhuis, M.,, Harder, W.   +8 more
core   +2 more sources

Long-Term Cholic Acid Therapy in Zellweger Spectrum Disorders

Case Reports in Gastroenterology, 2018
Zellweger spectrum disorders (ZSDs), a subgroup of peroxisomal biogenesis disorders, have a generalized defect in peroxisome function. Liver disease in ZSDs has been linked to accumulation of C27-bile acid intermediates due to the lack of peroxisomal β ...
James E. Heubi, Kenneth D.R. Setchell, Kevin E. Bove   +2 more
doaj   +1 more source

Pex13 inactivation in the mouse disrupts peroxisome biogenesis and leads to a Zellweger syndrome phenotype [PDF]

, 2002
Zellweger syndrome is the archetypical peroxisome biogenesis disorder and is characterized by defective import of proteins into the peroxisome, leading to peroxisomal metabolic dysfunction and widespread tissue pathology.
Kay, Graham F., Crane, Denis I., C. Paton, Barbara, Maxwell, M., Crane, D., Paterson, Carol, Kay, G., Bjorkman, J., Paterson, C., Nguyen, Tam, Sharp, P., Finnie, J., Nguyen, T., F. Kay, Graham, Paton, B., Maxwell, Megan, Tonks, I., Sharp, Peter, Crane, Denis, Paton, Barbara C., Bjorkman, Jonas, Finnie, John, Tonks, Ian   +22 more
core   +1 more source

Peroxisomal disorders: The single peroxisomal enzyme deficiencies [PDF]

, 2006
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 ...
Waterham, Hans R., Wanders, Ronald J. A., Wanders, Ronald J.A.   +2 more
core   +1 more source

Biochemical and clinical profiles of 52 Tunisian patients affected by Zellweger syndrome

Pediatrics and Neonatology, 2017
Background: Zellweger syndrome (ZS) is a peroxisome biogenesis disorder attributed to a mutation of the PEX genes family. The incidence of this disease in Africa and the Arab world remains unknown.
Fahmi Nasrallah, Wiem Zidi, Moncef Feki, Samia Kacem, Neji Tebib, Naziha Kaabachi   +5 more
doaj   +1 more source

Genotype–Phenotype Correlations of Monoallelic PFIC Variants in Pediatric Liver Disease: A Multicenter Retrospective Cohort Study

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Progressive familial intrahepatic cholestasis (PFIC) is classically caused by biallelic pathogenic variants, yet monoallelic variants of uncertain significance (VUS) in PFIC‐associated genes are increasingly identified in children with cholestasis, creating diagnostic uncertainty.
Brett J. Hoskins, Tiziano Pramparo, Chaowapong Jarasvaraparn, Michael J. Wilsey, Voytek Slowik, Lakshmi Kunam, Janis M. Stoll, Ruben E. Quiros‐Tejeira, Simon Lam, Wikrom Karnsakul   +9 more
wiley   +1 more source