8,338 results match your criteria Peroxisomal Disorders


Case report of dysregulation of primary bile acid synthesis in a family with X-linked adrenoleukodystrophy.

Medicine (Baltimore) 2018 Dec;97(49):e13353

Department of Clinical Biochemistry, Jagiellonian University Medical College, Krakow, Poland.

Rationale: X-linked adrenoleukodystrophy (X-ALD) is a rare disorder caused by mutations in the ABCD1 gene, coding for peroxisomal membrane transporter adrenoleukodystrophy (ALD) protein. The disease is characterized by accumulation of very long chain fatty acids (VLCFAs) in tissues. Adult adrenomyeloneuropathy (AMN) and the cerebral inflammatory form of ALD are the main phenotypes presenting various symptoms. Read More

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December 2018

Augmenter of liver regeneration protein deficiency promotes hepatic steatosis by inducing oxidative stress and microRNA-540 expression.

FASEB J 2018 Dec 12:fj201802015R. Epub 2018 Dec 12.

Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Levels of augmenter of liver regeneration (ALR), a multifunctional protein, are reduced in steatohepatitis. ALR depletion from ALR /Alb-Cre [ALR-L-knockout (KO)] mouse causes robust steatosis and apoptosis of hepatocytes, and pericellular fibrosis between 1 and 2 wk postbirth. Steatosis regresses by 4 wk upon reappearance of ALR-expressing hepatocytes. Read More

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December 2018

Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4.

FASEB J 2018 Dec 12:fj201801498R. Epub 2018 Dec 12.

Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

Peroxisomes are essential organelles for the specialized oxidation of a wide variety of fatty acids, but they are also able to degrade fatty acids that are typically handled by mitochondria. Using a combination of pharmacological inhibition and clustered regularly interspaced short palindromic repeats (CRISPR)-CRISPR associated protein 9 genome editing technology to simultaneously manipulate peroxisomal and mitochondrial fatty acid β-oxidation (FAO) in HEK-293 cells, we identified essential players in the metabolic crosstalk between these organelles. Depletion of carnitine palmitoyltransferase (CPT)2 activity through pharmacological inhibition or knockout (KO) uncovered a significant residual peroxisomal oxidation of lauric and palmitic acid, leading to the production of peroxisomal acylcarnitine intermediates. Read More

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December 2018
4 Reads

Miro1 - the missing link to peroxisome motility.

Commun Integr Biol 2018 12;11(4):e1526573. Epub 2018 Oct 12.

Biosciences, University of Exeter, Exeter, UK.

Peroxisomes are ubiquitous, highly dynamic, multifunctional compartments in eukaryotic cells, which perform key roles in cellular lipid metabolism and redox balance. Like other membrane-bound organelles, peroxisomes must move in the cellular landscape to perform localized functions, interact with other organelles and to properly distribute during cell division. However, our current knowledge of peroxisome motility in mammalian cells is still very limited. Read More

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October 2018

Dynamin-related protein 1 has membrane constricting and severing abilities sufficient for mitochondrial and peroxisomal fission.

Nat Commun 2018 12 7;9(1):5239. Epub 2018 Dec 7.

Department of Biochemistry and Molecular Biology, Monash Biomedicine Discovery Institute, Monash University, 3800, Melbourne, Australia.

Dynamin-related protein 1 (Drp1) is essential for mitochondrial and peroxisomal fission. Recent studies propose that Drp1 does not sever but rather constricts mitochondrial membranes allowing dynamin 2 (Dnm2) to execute final scission. Here, we report that unlike Drp1, Dnm2 is dispensable for peroxisomal and mitochondrial fission, as these events occurred in Dnm2 knockout cells. Read More

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December 2018

Peroxisome biogenesis, membrane contact sites, and quality control.

EMBO Rep 2018 Dec 10. Epub 2018 Dec 10.

Section of Molecular Biology, Division of Biological Sciences, University of California, San Diego, CA, USA

Peroxisomes are conserved organelles of eukaryotic cells with important roles in cellular metabolism, human health, redox homeostasis, as well as intracellular metabolite transfer and signaling. We review here the current status of the different co-existing modes of biogenesis of peroxisomal membrane proteins demonstrating the fascinating adaptability in their targeting and sorting pathways. While earlier studies focused on peroxisomes as autonomous organelles, the necessity of the ER and potentially even mitochondria as sources of peroxisomal membrane proteins and lipids has come to light in recent years. Read More

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December 2018

Peroxisome biogenesis deficiency attenuates the BDNF-TrkB pathway-mediated development of the cerebellum.

Life Sci Alliance 2018 Dec 3;1(6):e201800062. Epub 2018 Dec 3.

Division of Organelle Homeostasis, Medical Institute of Bioregulation, Kyushu University, Fukuoka, Japan.

Peroxisome biogenesis disorders (PBDs) manifest as neurological deficits in the central nervous system, including neuronal migration defects and abnormal cerebellum development. However, the mechanisms underlying pathogenesis remain enigmatic. Here, to investigate how peroxisome deficiency causes neurological defects of PBDs, we established a new PBD model mouse defective in peroxisome assembly factor Pex14p, termed mouse. Read More

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December 2018
1 Read

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

Case Rep Gastroenterol 2018 Sep-Dec;12(3):661-670. Epub 2018 Nov 21.

Division of Pediatric Gastroenterology, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.

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 C-bile acid intermediates to form primary C-bile acids, which prevents normal physiologic feedback and leads to accumulation of hepatotoxic bile acid intermediates. Primary bile acid therapy, oral cholic acid (CA), as adjunctive treatment for ZSDs, restores physiologic feedback inhibition on bile acid synthesis and inhibits formation of hepatotoxic bile acid intermediates. Read More

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November 2018
2 Reads

17B-hydroxysteroid dehydrogenases as acyl thioester metabolizing enzymes.

Mol Cell Endocrinol 2018 Nov 30. Epub 2018 Nov 30.

Faculty of Biochemistry and Molecular Medicine, University of Oulu, Oulu, Finland.

17β-Hydroxysteroid dehydrogenases (HSD17B) catalyze the oxidation/reduction of 17β-hydroxy/keto group in position C17 in C18- and C19 steroids. Most HSD17Bs are also catalytically active with substrates other than steroids. A subset of these enzymes is able to process thioesters of carboxylic acids. Read More

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November 2018

Peroxisome-derived lipids regulate adipose thermogenesis by mediating cold-induced mitochondrial fission.

J Clin Invest 2018 Dec 4. Epub 2018 Dec 4.

Peroxisomes perform essential functions in lipid metabolism, including fatty acid oxidation and plasmalogen synthesis. Here, we describe a role for peroxisomal lipid metabolism in mitochondrial dynamics in brown and beige adipocytes. Adipose tissue peroxisomal biogenesis was induced in response to cold exposure through activation of the thermogenic co-regulator PRDM16. Read More

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December 2018

Gene therapy for lysosomal storage diseases and peroxisomal diseases.

Authors:
Toya Ohashi

J Hum Genet 2018 Nov 29. Epub 2018 Nov 29.

Division of Gene Therapy, Research Center for Medical Sciences/Department of Pediatrics, The Jikei University School of Medicine, Tokyo, Japan.

Gene therapies for lysosomal storage diseases (LSD) and peroxisomal diseases (PD) are rapidly advancing. Most LSDs and PDs are characterized by brain involvement, prompting the development of therapies targeting the brain. There are two types of gene therapy for brain involvement in LSD and PD, i. Read More

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November 2018
1 Read

Effect of mcl-PHA synthesis in flax on plant mechanical properties and cell wall composition.

Transgenic Res 2018 Nov 27. Epub 2018 Nov 27.

Department of Biotechnology and Food Microbiology, Faculty of Biotechnology and Food Sciences, Wrocław University of Environmental and Life Sciences, Chełmońskiego St. 37, 51-630, Wrocław, Poland.

The high demand for new biomaterials makes synthesis of polyhydroxyalkanoates (PHA) in plants an interesting and desirable achievement. Production of polymers in plants is an example of application of biotechnology for improving the properties of plants, e.g. Read More

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November 2018
2 Reads

A newly identified mutation in the gene is associated with a milder type of Zellweger spectrum disorder.

Cold Spring Harb Mol Case Stud 2018 Nov 16. Epub 2018 Nov 16.

Medical Institute of Bioregulation, Kyushu University;

Using clinical exome sequencing (ES), we identified an autosomal recessive missense variant, c.153C>A (p.F51L), in the peroxisome biogenesis factor 26 gene () in a 19-year old female who was referred for moderate to severe hearing loss. Read More

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November 2018
3 Reads

The intrinsically disordered nature of the peroxisomal protein translocation machinery.

FEBS J 2018 Nov 16. Epub 2018 Nov 16.

Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Portugal.

Despite having a membrane that is impermeable to all but the smallest of metabolites, peroxisomes acquire their newly synthesized (cytosolic) matrix proteins in an already folded conformation. In some cases, even oligomeric proteins have been reported to translocate the organelle membrane. The protein sorting machinery that accomplishes this feat must be rather flexible and, unsurprisingly, several of its key components have large intrinsically disordered domains. Read More

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November 2018
1 Read

Membrane topologies of PEX13 and PEX14 provide new insights on the mechanism of protein import into peroxisomes.

FEBS J 2018 Nov 10. Epub 2018 Nov 10.

Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Portugal.

PEX13 and PEX14 are two core components of the so-called peroxisomal docking/translocation module, the transmembrane hydrophilic channel through which newly synthesized peroxisomal proteins are translocated into the organelle matrix. The two proteins interact with each other and with PEX5, the peroxisomal matrix protein shuttling receptor, through relatively well characterized domains. However, the topologies of these membrane proteins are still poorly defined. Read More

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November 2018
3 Reads

The deubiquitination of the PTS1-import receptor Pex5p is required for peroxisomal matrix protein import.

Biochim Biophys Acta Mol Cell Res 2018 Nov 6;1866(2):199-213. Epub 2018 Nov 6.

Biochemie Intrazellulärer Transportprozesse, Ruhr-Universität Bochum, 44801 Bochum, Germany. Electronic address:

Peroxisomal biogenesis depends on the correct import of matrix proteins into the lumen of the organelle. Most peroxisomal matrix proteins harbor the peroxisomal targeting-type 1 (PTS1), which is recognized by the soluble PTS1-receptor Pex5p in the cytosol. Pex5p ferries the PTS1-proteins to the peroxisomal membrane and releases them into the lumen. Read More

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November 2018

Onsite GTP fuelling via DYNAMO1 drives division of mitochondria and peroxisomes.

Nat Commun 2018 11 6;9(1):4634. Epub 2018 Nov 6.

Division of Organelle Homeostasis, Medical Institute of Bioregulation, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.

Mitochondria and peroxisomes proliferate by division. During division, a part of their membrane is pinched off by constriction of the ring-shaped mitochondrial division (MD) and peroxisome-dividing (POD) machinery. This constriction is mediated by a dynamin-like GTPase Dnm1 that requires a large amount of GTP as an energy source. Read More

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November 2018
10 Reads

A bacteria-derived tail anchor localizes to peroxisomes in yeast and mammalian cells.

Sci Rep 2018 Nov 6;8(1):16374. Epub 2018 Nov 6.

Institute of Biotechnology, Helsinki Institute of Life Science, University of Helsinki, 00014, Helsinki, Finland.

Prokaryotes can provide new genetic information to eukaryotes by horizontal gene transfer (HGT), and such transfers are likely to have been particularly consequential in the era of eukaryogenesis. Since eukaryotes are highly compartmentalized, it is worthwhile to consider the mechanisms by which newly transferred proteins might reach diverse organellar destinations. Toward this goal, we have focused our attention upon the behavior of bacteria-derived tail anchors (TAs) expressed in the eukaryote Saccharomyces cerevisiae. Read More

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November 2018
5 Reads

Unusual Clinical Course and Imaging of D-Bifunctional Protein Deficiency, a Rare Leukodystrophy.

Pediatr Neurol 2018 Sep 21. Epub 2018 Sep 21.

University of Mississippi Medical Center, Jackson, Mississippi.

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September 2018
2 Reads

Calcium-independent phospholipase Aγ (iPLAγ) and its roles in cellular functions and diseases.

Biochim Biophys Acta Mol Cell Biol Lipids 2018 Nov 1. Epub 2018 Nov 1.

Division of Health Chemistry, Department of Healthcare and Regulatory Sciences, School of Pharmacy, Showa University, Tokyo 142-8555, Japan.

Calcium-independent phospholipase Aγ (iPLAγ)/patatin-like phospholipase domain-containing lipase 8 (PNPLA8) is one of the iPLA enzymes, which do not require Ca ion for their activity. iPLAγ is a membrane-bound enzyme with unique features, including the utilization of four distinct translation initiation sites and the presence of mitochondrial and peroxisomal localization signals. This enzyme is preferentially distributed in the mitochondria and peroxisomes and is thought to be responsible for the maintenance of lipid homeostasis in these organelles. Read More

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November 2018

Distinct Roles for Peroxisomal Targeting Signal Receptors Pex5 and Pex7 in .

Genetics 2018 Nov 2. Epub 2018 Nov 2.

University of Alberta

Peroxisomes are ubiquitous membrane-enclosed organelles involved in lipid processing and reactive oxygen detoxification. Mutations in human peroxisome biogenesis genes (, or ) cause developmental disabilities and often early death. Pex5 and Pex7 are receptors that recognize different peroxisomal targeting signals called PTS1 and PTS2, respectively, and traffic proteins to the peroxisomal matrix. Read More

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November 2018
2 Reads

Secreted d-aspartate oxidase functions in C. elegans reproduction and development.

FEBS J 2018 Nov 2. Epub 2018 Nov 2.

Laboratory of Biomolecular Science, Graduate School of Pharmaceutical Sciences, Kitasato University, Shirokane, Minato-ku, Japan.

d-Aspartate oxidase (DDO) is a degradative enzyme that acts stereospecifically on free acidic D-amino acids such as d-aspartate and d-glutamate. d-Aspartate plays an important role in regulating neurotransmission, developmental processes, hormone secretion, and reproductive functions in mammals. In contrast, the physiological role of d-glutamate in mammals remains unclear. Read More

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November 2018
8 Reads

A Role for RNS in the Communication of Plant Peroxisomes with Other Cell Organelles?

Subcell Biochem 2018 ;89:473-493

Group of Antioxidants, Free Radicals and Nitric Oxide in Biotechnology, Food and Agriculture, Department of Biochemistry, Cell and Molecular Biology of Plants, Estación Experimental del Zaidín, CSIC, Profesor Albareda 1, 18008, Granada, Spain.

Plant peroxisomes are organelles with a very active participation in the cellular regulation of the metabolism of reactive oxygen species (ROS). However, during the last two decades peroxisomes have been shown to be also a relevant source of nitric oxide (NO) and other related molecules designated as reactive nitrogen species (RNS). ROS and RNS have been mainly associated to nitro-oxidative processes; however, some members of these two families of molecules such as HO, NO or S-nitrosoglutathione (GSNO) are also involved in the mechanism of signaling processes mainly through post-translational modifications. Read More

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January 2018
2 Reads

Cell Death or Survival Against Oxidative Stress.

Subcell Biochem 2018 ;89:463-471

Medical Institute of Bioregulation, Kyushu University, 812-8582, Higashi-ku, Fukuoka, Japan.

Peroxisomes contain anabolic and catabolic enzymes including oxidases that produce hydrogen peroxide as a by-product. Peroxisomes also contain catalase to metabolize hydrogen peroxide. It has been recognized that catalase is localized to cytosol in addition to peroxisomes. Read More

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January 2018
3 Reads

Peroxisomes and Cellular Oxidant/Antioxidant Balance: Protein Redox Modifications and Impact on Inter-organelle Communication.

Subcell Biochem 2018 ;89:435-461

Department of Cellular and Molecular Medicine, KU Leuven, Herestraat 49, Box 601, 3000, Louvain, Belgium.

Disturbances in cellular redox balance have been associated with pro-aging mechanisms and increased risk for various chronic disease states. Multiple lines of evidence indicate that peroxisomes are central players in cellular redox metabolism. Nevertheless, the potential role of this organelle as intracellular redox signaling platform has been largely overlooked for a long time. Read More

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January 2018
7 Reads

Peroxisome Mitochondria Inter-relations in Plants.

Subcell Biochem 2018 ;89:417-433

Laboratory of Plant Development and Interactions, Department of Molecular and Cellular Biology, University of Guelph, 50 Stone Road, Guelph, ON, N1G2W1, Canada.

A large amount of ultrastructural, biochemical and molecular analysis indicates that peroxisomes and mitochondria not only share the same subcellular space but also maintain considerable overlap in their proteins, responses and functions. Recent approaches using imaging of fluorescent proteins targeted to both organelles in living plant cells are beginning to show the dynamic nature of their interactivity. Based on the observations of living cells, mitochondria respond rapidly to stress by undergoing fission. Read More

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January 2018

Multi-localized Proteins: The Peroxisome-Mitochondria Connection.

Subcell Biochem 2018 ;89:383-415

Biosciences, University of Exeter, Exeter, EX4 4QD, UK.

Peroxisomes and mitochondria are dynamic, multifunctional organelles that play pivotal cooperative roles in the metabolism of cellular lipids and reactive oxygen species. Their functional interplay, the "peroxisome-mitochondria connection", also includes cooperation in anti-viral signalling and defence, as well as coordinated biogenesis by sharing key division proteins. In this review, we focus on multi-localised proteins which are shared by peroxisomes and mitochondria in mammals. Read More

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January 2018
4 Reads

Characterization of Peroxisomal Regulation Networks.

Subcell Biochem 2018 ;89:367-382

Center for Infectious Disease Research, 307 Westlake Avenue North, Suite 500, Seattle, WA, 98109-5219, USA.

Peroxisome proliferation involves signal recognition and computation by molecular networks that direct molecular events of gene expression, metabolism, membrane biogenesis, organelle proliferation, protein import, and organelle inheritance. Peroxisome biogenesis in yeast has served as a model system for exploring the regulatory networks controlling this process. Yeast is an outstanding model system to develop tools and approaches to study molecular networks and cellular responses and because the mechanisms of peroxisome biogenesis and key aspects of the transcriptional regulatory networks are remarkably conserved from yeast to humans. Read More

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January 2018
3 Reads

The Proteome of Fruit Peroxisomes: Sweet Pepper (Capsicum annuum L.) as a Model.

Subcell Biochem 2018 ;89:323-341

Group of Antioxidants, Free Radicals and Nitric Oxide in Biotechnology, Food and Agriculture, Estación Experimental del Zaidín, CSIC, Profesor Albareda 1, 18008, Granada, Spain.

Despite of their economical and nutritional interest, the biology of fruits is still little studied in comparison with reports of other plant organs such as leaves and roots. Accordingly, research at subcellular and molecular levels is necessary not only to understand the physiology of fruits, but also to improve crop qualities. Efforts addressed to gain knowledge of the peroxisome proteome and how it interacts with the overall metabolism of fruits will provide tools to be used in breeding strategies of agricultural species with added value. Read More

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January 2018
8 Reads

Unraveling of the Structure and Function of Peroxisomal Protein Import Machineries.

Subcell Biochem 2018 ;89:299-321

Department of Systems Biochemistry, Faculty of Medicine, Institute of Biochemistry and Pathobiochemistry, Ruhr University Bochum, Universitätsstr. 150, 44801, Bochum, Germany.

Peroxisomes are dynamic organelles of eukaryotic cells performing a wide range of functions including fatty acid oxidation, peroxide detoxification and ether-lipid synthesis in mammals. Peroxisomes lack their own DNA and therefore have to import proteins post-translationally. Peroxisomes can import folded, co-factor bound and even oligomeric proteins. Read More

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January 2018
3 Reads

Identification of Peroxisomal Protein Complexes with PTS Receptors, Pex5 and Pex7, in Mammalian Cells.

Subcell Biochem 2018 ;89:287-298

Medical Institute of Bioregulation, Kyushu University, Higashi-ku, Fukuoka, 812-8582, Japan.

Pex5 and Pex7 are cytosolic receptors for peroxisome targeting signal type-1 (PTS1) and type-2 (PTS2), respectively, and play a pivotal role in import of peroxisomal matrix proteins. Recent advance in mass spectrometry analysis has facilitated comprehensive analysis of protein-protein interaction network by a combination with immunoprecipitation or biochemical purification. In this chapter, we introduce several findings obtained by these methods applied to mammalian cells. Read More

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January 2018

Using Pull Down Strategies to Analyze the Interactome of Peroxisomal Membrane Proteins in Human Cells.

Subcell Biochem 2018 ;89:261-285

Institute of Biochemistry and Pathobiochemistry, Ruhr-University Bochum, 44780, Bochum, Germany.

Different pull-down strategies were successfully applied to gain novel insight into the interactome of human membrane-associated proteins. Here, we compare the outcome, efficiency and potential of pull-down strategies applied to human peroxisomal membrane proteins. Stable membrane-bound protein complexes can be affinity-purified from genetically engineered human cells or subfractions thereof after detergent solubilization, followed by size exclusion chromatography and analysis by mass spectrometry (MS). Read More

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January 2018
4 Reads

Evolution of the Peroxisomal Proteome.

Authors:
Toni Gabaldón

Subcell Biochem 2018 ;89:221-233

Centre for Genomic Regulation (CRG), The Barcelona Institute of Science and Technology (BIST), Dr. Aiguader, 88, 08003, Barcelona, Spain.

Peroxisomes are single-membrane bound intracellular organelles that can be found in organisms across the tree of eukaryotes, and thus are likely to derive from an ancestral peroxisome in the last eukaryotic common ancestor (LECA). Yet, peroxisomes in different lineages can present a large diversity in terms of their metabolic capabilities, which reflects a highly variable proteomic content. Theories on the evolutionary origin of peroxisomes have shifted in the last decades from scenarios involving an endosymbiotic origin, similar to those of mitochondria and plastids, towards hypotheses purporting an endogenous origin from within the endomembrane system. Read More

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January 2018
1 Read

Predicting Peroxisomal Targeting Signals to Elucidate the Peroxisomal Proteome of Mammals.

Authors:
Markus Kunze

Subcell Biochem 2018 ;89:157-199

Department of Pathobiology of the Nervous System, Center for Brain Research, Medical University of Vienna, Vienna, Austria.

Peroxisomes harbor a plethora of proteins, but the peroxisomal proteome as the entirety of all peroxisomal proteins is still unknown for mammalian species. Computational algorithms can be used to predict the subcellular localization of proteins based on their amino acid sequence and this method has been amply used to forecast the intracellular fate of individual proteins. However, when applying such algorithms systematically to all proteins of an organism the prediction of its peroxisomal proteome in silico should be possible. Read More

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January 2018
4 Reads

The Obvious and the Hidden: Prediction and Function of Fungal Peroxisomal Matrix Proteins.

Subcell Biochem 2018 ;89:139-155

Department of Biology, Philipps-Universität Marburg, Marburg, Germany.

Fungal peroxisomes are characterized by a number of specific biological functions. To understand the physiology and biochemistry of these organelles knowledge of the proteome content is crucial. Here, we address different strategies to predict peroxisomal proteins by bioinformatics approaches. Read More

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January 2018
1 Read

Prediction of Peroxisomal Matrix Proteins in Plants.

Subcell Biochem 2018 ;89:125-138

KIIT School of Biotechnology, Campus XI, KIIT University, Bhubaneswar, 751024, India.

Our knowledge of the proteome of plant peroxisomes is far from being complete, and the functional complexity and plasticity of this cell organelle are amazingly high particularly in plants, as exemplified by the model species Arabidopsis thaliana. Plant-specific peroxisome functions that have been uncovered only recently include, for instance, the participation of peroxisomes in phylloquinone and biotin biosynthesis. Experimental proteome studies have been proved very successful in defining the proteome of Arabidopsis peroxisomes but this approach also faces significant challenges and limitations. Read More

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January 2018

Fungal Peroxisomes Proteomics.

Subcell Biochem 2018 ;89:67-83

Molecular Cell Biology, Groningen Biomolecular Sciences and Biotechnology Institute, University of Groningen, 9747AG, Groningen, The Netherlands.

Peroxisomes in fungi are involved in a huge number of different metabolic processes. In addition, non-metabolic functions have also been identified. The proteins that are present in a particular peroxisome determine its metabolic function, whether they are the matrix localized enzymes of the different metabolic pathways or the membrane proteins involved in transport of metabolites across the peroxisomal membrane. Read More

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January 2018
1 Read

Defining the Mammalian Peroxisomal Proteome.

Subcell Biochem 2018 ;89:47-66

Faculty of Biology and BIOSS Centre for Biological Signaling Studies, Biochemistry and Functional Proteomics, Institute for Biology II, University of Freiburg, 79104, Freiburg, Germany.

The current view on peroxisomes has changed dramatically from being human cell oddities to vital organelles that host several key metabolic pathways. To fulfil over 50 different enzymatic functions, human peroxisomes host either unique peroxisomal proteins or dual-localized proteins. The identification and characterization of the complete peroxisomal proteome in humans is important for diagnosis and treatment of patients with peroxisomal disorders as well as for uncovering novel peroxisomal functions and regulatory modules. Read More

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January 2018
1 Read

Proteome of Plant Peroxisomes.

Subcell Biochem 2018 ;89:3-45

MSU-Department of Energy Plant Research Laboratory, Michigan State University, East Lansing, MI, 48824, USA.

Plant peroxisomes are required for a number of fundamental physiological processes, such as primary and secondary metabolism, development and stress response. Indexing the dynamic peroxisome proteome is prerequisite to fully understanding the importance of these organelles. Mass Spectrometry (MS)-based proteome analysis has allowed the identification of novel peroxisomal proteins and pathways in a relatively high-throughput fashion and significantly expanded the list of proteins and biochemical reactions in plant peroxisomes. Read More

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January 2018
1 Read

Chemically monoubiquitinated PEX5 binds to the components of the peroxisomal docking and export machinery.

Sci Rep 2018 Oct 30;8(1):16014. Epub 2018 Oct 30.

Interfakultäres Institut für Biochemie (IFIB), Universität Tübingen, Tübingen, Germany.

Peroxisomal matrix proteins contain either a peroxisomal targeting sequence 1 (PTS1) or a PTS2 that are recognized by the import receptors PEX5 and PEX7, respectively. PEX5 transports the PTS1 proteins and the PEX7/PTS2 complex to the docking translocation module (DTM) at the peroxisomal membrane. After cargo release PEX5 is monoubiquitinated and extracted from the peroxisomal membrane by the receptor export machinery (REM) comprising PEX26 and the AAA ATPases PEX1 and PEX6. Read More

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October 2018

Isoform-specific domain organization determines conformation and function of the peroxisomal biogenesis factor PEX26.

Biochim Biophys Acta Mol Cell Res 2018 Oct 23. Epub 2018 Oct 23.

University Children's Research@Kinder-UKE, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany; Children's Hospital, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany. Electronic address:

Peroxisomal biogenesis factor PEX26 is a membrane anchor for the multi-subunit PEX1-PEX6 protein complex that controls ubiquitination and dislocation of PEX5 cargo receptors for peroxisomal matrix protein import. PEX26 associates with the peroxisomal translocation pore via PEX14 and a splice variant (PEX26Δex5) of unknown function has been reported. Here, we demonstrate PEX26 homooligomerization mediated by two heptad repeat domains adjacent to the transmembrane domain. Read More

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October 2018
4 Reads

Zellweger spectrum disorder patient-derived fibroblasts with the PEX1-Gly843Asp allele recover peroxisome functions in response to flavonoids.

J Cell Biochem 2018 Oct 26. Epub 2018 Oct 26.

Department of Human Genetics, McGill University, Montreal, Quebec, Canada.

Zellweger spectrum disorder (ZSD) results from biallelic mutations in PEX genes required for peroxisome biogenesis. PEX1-G843D is a common hypomorphic allele in the patient population that is associated with milder disease. In prior work using a PEX1-G843D/null patient fibroblast line expressing a green fluorescent protein (GFP) reporter with a peroxisome-targeting signal (GFP-PTS1), we demonstrated that treatments with the chemical chaperone betaine and flavonoid acacetin diacetate recovered peroxisome functions. Read More

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October 2018
11 Reads

Roles of extrahepatic lipolysis and the disturbance of hepatic fatty acid metabolism in TNF-α -induced hepatic steatosis.

Toxicology 2018 Oct 22. Epub 2018 Oct 22.

Institute of Toxicology, School of Public Health, Shandong University, 44 Wenhua West Road, Shandong Province, Jinan City, 250012, PR China. Electronic address:

Our previous study showed that both Kupffer cell eliminator (GdCl) and tumor necrosis factor α (TNF-α) receptor antagonist (etanercept) could partially attenuate binge drinking-induced liver steatosis. Herein, we extended the study by directly investigating the roles of TNF-α on the hepatic fat levels in mice and in HepG2 cells, and explored the underlying mechanisms. SPF male ICR mice were exposed to TNF-α (0. Read More

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October 2018
4 Reads

Intrathecal Adeno-Associated Virus Vector-mediated Gene Delivery for Adrenomyeloneuropathy.

Hum Gene Ther 2018 Oct 25. Epub 2018 Oct 25.

Massachusetts General Hospital, Neurology , 55 Fruit Street , Boston, Massachusetts, United States , 02114 ;

Mutations in the gene encoding the peroxisomal ATP binding cassette transporter (ABCD1) cause elevations in very long chain fatty acids (VLCFA) and the neurodegenerative disease adrenoleukodystrophy (ALD). In most adults, this manifests as the spinal cord axonopathy, adrenomyeloneuropathy (AMN). A challenge in virus-based gene therapy in AMN is how to achieve functional gene correction to the entire spinal cord while minimizing leakage into the systemic circulation, which could contribute to toxicity. Read More

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October 2018
2 Reads

Deletion of Lymphangiogenic and Angiogenic Growth Factor VEGF-D Leads to Severe Hyperlipidemia and Delayed Clearance of Chylomicron Remnants.

Arterioscler Thromb Vasc Biol 2018 Oct;38(10):2327-2337

From the A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio (A.T., T.V., S.K., K.H., H.N., H.L., M.U.K., S.Y.-H.).

Objective- Dyslipidemia is one of the key factors behind coronary heart disease. Blood and lymphatic vessels play pivotal roles in both lipoprotein metabolism and development of atherosclerotic plaques. Recent studies have linked members of VEGF (vascular endothelial growth factor) family to lipid metabolism, but the function of VEGF-D has remained unexplored. Read More

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October 2018
5 Reads

Photometric screens identified Arabidopsis peroxisome proteins that impact photosynthesis under dynamic light conditions.

Plant J 2018 Oct 23. Epub 2018 Oct 23.

Department of Energy Plant Research Laboratory, Michigan State University, East Lansing, MI, 48824, USA.

Plant peroxisomes function collaboratively with other subcellular organelles, such as chloroplasts and mitochondria, in several metabolic processes. To comprehensively investigate the impact of peroxisomal function on photosynthesis, especially under conditions that are more relevant to natural environments, a systematic screen of over 150 Arabidopsis mutants of genes encoding peroxisomal proteins was conducted using the automated Dynamic Environment Photosynthesis Imager (DEPI). Dynamic and high-light (HL) conditions triggered significant photosynthetic defects in a subset of the mutants, including those of photorespiration (PR) and other peroxisomal processes, some of which may also be related to PR. Read More

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October 2018
4 Reads

Light-induced intracellular hydrogen peroxide generation through genetically encoded photosensitizer KillerRed-SOD1.

Free Radic Res 2018 Oct 23:1-211. Epub 2018 Oct 23.

a Institute of Clinical Biochemistry , Hannover Medical School , Hannover , Germany.

Hydrogen peroxide plays an important role in various biological processes in numerous organisms. Depending on the concentration and the distribution within the cell, it can act as stressor or redox signalling molecule. To analyse the effects of HO and its diffusion within the cell we developed the new genetically encoded photosensitizer KillerRed-SOD1 which enables a light-induced spatially and temporally controlled generation of HO in living cells. Read More

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October 2018
5 Reads

The C Terminus of Rotavirus VP4 Protein Contains an Actin Binding Domain Which Requires Cooperation with the Coiled-Coil Domain for Actin Remodeling.

J Virol 2019 Jan 10;93(1). Epub 2018 Dec 10.

Sorbonne Universités, UPMC University Paris 06, École Normale Supérieure, PSL Research University, CNRS, INSERM U1157, APHP, Laboratoire des Biomolécules (LBM), Paris, France

The interactions between viruses and actin cytoskeleton have been widely studied. We showed that rotaviruses remodel microfilaments in intestinal cells and demonstrated that this was due to the VP4 spike protein. Microfilaments mainly occur in the apical domain of infected polarized enterocytes and favor the polarized apical exit of viral progeny. Read More

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January 2019
7 Reads

Transcriptome Comparative Analysis of Salt Stress Responsiveness in Chrysanthemum (Dendranthema grandiflorum) Roots by Illumina- and Single-Molecule Real-Time-Based RNA Sequencing.

DNA Cell Biol 2018 Oct 16. Epub 2018 Oct 16.

Department of Ornamental Horticulture, Sichuan Agricultural University , Chengdu, People's Republic of China .

Salt response has long been considered a polygenic-controlled character in plants. Under salt stress conditions, plants respond by activating a great amount of proteins and enzymes. To develop a better understanding of the molecular mechanism and screen salt responsive genes in chrysanthemum under salt stress, we performed the RNA sequencing (RNA-seq) on both salt-processed chrysanthemum seedling roots and the control group, and gathered six cDNA databases eventually. Read More

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October 2018
2.060 Impact Factor

Neonatal punctate calcifications associated with maternal mixed connective tissue disorder (MCTD).

BMJ Case Rep 2018 Oct 12;2018. Epub 2018 Oct 12.

Neonatology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Chondrodysplasia punctate (CDP) is a rare group of disorders with both genetic and non-genetic underlying aetiologies. The genetic causes associated with CDP include peroxisomal disorders, type two mucolipidosis, type 3 mucopolysaccharidosis, GM1 gangliosidosis and chromosomal disorders. Peroxisomal disorders include deficiency of dihydroxyacetone phosphate acyltransferase, encoded by GNPAT, deficiency of the peroxisomal enzyme alkyl-dihydroxyacetone phosphate synthase, encoded by AGPS and Zellweger syndrome. Read More

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October 2018
6 Reads