Peroxisomal membrane ghosts in Zellweger syndrome--aberrant organelle assembly

Science
M J SantosP B Lazarow

Abstract

Peroxisomes are apparently missing in Zellweger syndrome; nevertheless, some of the integral membrane proteins of the organelle are present. Their distribution was studied by immunofluorescence microscopy. In control fibroblasts, peroxisomes appeared as small dots. In Zellweger fibroblasts, the peroxisomal membrane proteins were located in unusual empty membrane structures of larger size. These results suggest that the primary defect in this disease may be in the mechanism for import of matrix proteins.

Citations

Mar 15, 2000·Annals of Neurology·W B Rizzo
Dec 1, 1990·Annals of Neurology·M MacCollinM Beard
Jan 1, 1997·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·H R Waterham, J M Cregg
Jan 1, 1994·Journal of Inherited Metabolic Disease·B FournierB T Poll-The
Jan 1, 1993·Journal of Inherited Metabolic Disease·W M FrederiksR J Wanders
Jan 1, 1993·Virchows Archiv. A, Pathological Anatomy and Histopathology·J L HughesA Poulos
Dec 1, 1988·Journal of the Neurological Sciences·R J WandersJ M Tager
Aug 1, 1990·Journal of the Neurological Sciences·R J WandersJ M Tager
Jun 1, 1989·Biochimica Et Biophysica Acta·P Borst
Nov 1, 1995·Pediatric Neurology·A NakaiY Konishi
Nov 1, 1992·Free Radical Biology & Medicine·L A del RíoF J Corpas
Jul 25, 2000·Trends in Genetics : TIG·S J Gould, D Valle
Jan 1, 1993·Biology of the Cell·C CauseretM Bugaut
Oct 29, 1996·Biochimica Et Biophysica Acta·Y Elgersma, H F Tabak
Aug 2, 2003·Current Opinion in Cell Biology·Paul B Lazarow
Jan 9, 2001·Trends in Cell Biology·V I Titorenko, R A Rachubinski
Jul 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·R ErdmannW H Kunau
Sep 1, 1989·Proceedings of the National Academy of Sciences of the United States of America·L A AllenC R Raetz
Aug 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·G A KellerR A Hallewell
Nov 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·J AikawaG L Chen
Feb 1, 1993·The Journal of Cell Biology·M Wendland, S Subramani
May 1, 1994·The Journal of Cell Biology·A MotleyH Tabak

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Epigenetics Insights from Twin Studies

Find the latest research on epigenetics and twin studies here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Regulation of Vocal-Motor Plasticity

Dopaminergic projections to the basal ganglia and nucleus accumbens shape the learning and plasticity of motivated behaviors across species including the regulation of vocal-motor plasticity and performance in songbirds. Discover the latest research on the regulation of vocal-motor plasticity here.

Myocardial Stunning

Myocardial stunning is a mechanical dysfunction that persists after reperfusion of previously ischemic tissue in the absence of irreversible damage including myocardial necrosis. Here is the latest research.