A5071560474- Peroxisome Proliferator-Activated Receptors
- Economic and Social Issues
- Adipose Tissue and Metabolism
- Metabolism and Genetic Disorders
- Regional Development and Policy
- Social Policy and Reform Studies
- German Economic Analysis & Policies
- Cholesterol and Lipid Metabolism
- Lysosomal Storage Disorders Research
- Sociology and Education Studies
- Eicosanoids and Hypertension Pharmacology
- Immune cells in cancer
- Political Economy and Marxism
- Lipid metabolism and biosynthesis
- Drug Transport and Resistance Mechanisms
- Economic Theory and Institutions
- Labor Movements and Unions
- Experimental Behavioral Economics Studies
- RNA regulation and disease
- Neuroinflammation and Neurodegeneration Mechanisms
- Labor market dynamics and wage inequality
- RNA modifications and cancer
- Diverse Legal and Medical Studies
- European Monetary and Fiscal Policies
- RNA Research and Splicing
Medical University of Vienna
2016-2025
Bielefeld University
1979-2024
Advisory Board Company (United States)
2021-2024
Walter de Gruyter (Germany)
2022-2024
University of Kaiserslautern
2023-2024
Humboldt-Universität zu Berlin
2021-2022
Charité - Universitätsmedizin Berlin
2020-2022
University Hospital Regensburg
2022
Freie Universität Berlin
2021-2022
University of Mannheim
2003-2021
The peroxisome represents a ubiquitous single membrane-bound key organelle that executes various metabolic pathways such as fatty acid degradation by α- and β-oxidation, ether-phospholipid biosynthesis, metabolism of reactive oxygen species, detoxification glyoxylate in mammals. To fulfil this vast array functions, peroxisomes accommodate ∼50 different enzymes at least identified until now. Interest has been fueled the discovery group genetic diseases humans, which are caused either defect...
In its severe form, X-linked adrenoleukodystrophy (ALD) is a lethal neurologic disease of children, characterized by progressive cerebral demyelination and adrenal insufficiency. Associated with biochemical defect peroxisomal β-oxidation, very long-chain fatty acids (VLCFA) build up in tissues that have high turnover lipids, such as central nervous system (CNS) white matter, cortex, testis. Whether the abnormal accumulation VLCFA underlying cause or merely an associated marker unknown. ALD...
Pex5p is the receptor for peroxisomal targeting signal 1 (PTS1) that consists of a C-terminal tripeptide (consensus (S/A/C)(K/R/H)(L/M)). Hexadecapeptides recognized by from Homo sapiens and Saccharomyces cerevisiae were identified screening two-hybrid peptide library, ability peptides was demonstrated using green fluorescent protein as reporter. The PTS1 receptors in species-specific manner broad range tripeptides, these are reported herein. In addition, residues upstream influenced...
In Alzheimer's disease (AD), lipid alterations are present early during progression. As some of these point towards a peroxisomal dysfunction, we investigated peroxisomes in human postmortem brains obtained from the cohort-based, longitudinal Vienna-Transdanube Aging (VITA) study. Based on neuropathological Braak staging for AD one hemisphere, patients were grouped into three cohorts increasing severity (stages I-II, III-IV, and V-VI, respectively). Lipid analyses cortical regions other...
Dysferlin is a muscle protein involved in cell membrane repair and its deficiency associated with muscular dystrophy. We describe that dysferlin also expressed leaky endothelial cells. In the normal central nervous system (CNS), only present cells of circumventricular organs. inflamed CNS patients multiple sclerosis (MS) or animals experimental autoimmune encephalomyelitis, reactivity induced expression vascular leakage serum proteins. MS, not restricted to vessels inflammatory cuffs but...
X-linked adrenoleukodystrophy (X-ALD), an inherited peroxisomal disorder, is caused by mutations in the ABCD1 gene encoding ATP-binding cassette (ABC) transporter (adrenoleukodystrophy protein, ALDP). Biochemically, X-ALD characterized accumulation of very long-chain fatty acids and partially impaired β-oxidation. In this study, we used primary human fibroblasts from Zellweger syndrome patients to investigate β-oxidation defect. Our results show that degradation C26:0-CoA esters as severely...
Significance Although sequencing of the human genome was completed years ago, we still do not know about physiological significance thousands predicted proteins, particularly membrane proteins. On other hand, for approximately 100 enzymes, no coding gene is known even though their enzymatic reaction has been well characterized. In this work, assign one those proteins (transmembrane protein 189; TMEM189) to reactions with an uncharacterized (plasmanylethanolamine desaturase). This enzyme...
By consensus, the acyl-CoA synthetase (ACS) community, with advice of human and mouse genome nomenclature committees, has revised for mammalian long-chain synthetases. ACS is family root name, genes ACSs are termed ACSL1,3-6 Acsl1,3-6, respectively. Splice variants ACSL3, -4, -5, -6 cataloged. Suggestions naming other members nonmammalian synthetases made.
The fatty acid transport protein (FATP) family is a group of proteins that are predicted to be components specific trafficking pathways. In mammalian systems, six different isoforms have been identified, which function in the import exogenous acids or activation very long-chain acids. This has led controversy as whether these membrane-bound transporters acyl-CoA synthetases, activate concomitant with transport. yeast FATP orthologue, Fat1p, dual functional and required for both acids;...
Inherited defects in the peroxisomal ATP-binding cassette (ABC) transporter adrenoleukodystrophy protein (ALDP) lead to lethal disorder X-linked (X-ALD), for which no efficient treatment has been established so far. Three other ABC transporters currently are known: adrenoleukodystrophy-related (ALDRP), 70 kDa membrane (PMP70) and PMP70-related protein. By using transient stable overexpression of human cDNAs encoding ALDP its closest relative ALDRP, we could restore impaired β-oxidation...
P426L and I179S are the two most frequent mutations in juvenile adult metachromatic leukodystrophy (late-onset MLD), which, contrast to infantile MLD, show marked phenotypic heterogeneity.To search for genotype-phenotype correlations late-onset MLD.The authors reviewed clinical course of 22 patients homozygous mutation vs 20 heterozygous I179S, which second arylsulfatase A (ASA) had also been determined.P426L homozygotes principally presented with progressive gait disturbance caused by...
A real-effort experiment is investigated in which supervisors have to rate the performance of individual workers who turn receive a bonus payment based on these ratings. We compare baseline treatment are not restricted their rating behavior forced distribution system they assign differentiated grades. find that productivity significantly higher under by about 6% 12%. However, effects less clear cut when participants prior experience with condition. Moreover, becomes detrimental access simple...
Abstract Because α‐synuclein (Snca) has a role in brain lipid metabolism, we determined the impact that Snca deletion had on whole composition. We analysed masses of individual phospholipid (PL) classes and neutral mass as well PL acyl chain composition brains from wild‐type ‐/‐ mice. Although total was not altered, cardiolipin phosphatidylglycerol decreased 16% 27%, respectively, In addition, no changes were observed plasmalogen or polyphosphoinositide mass. ethanolamine...
Ethanolamine plasmalogens constitute a group of ether glycerophospholipids that, due to their unique biophysical and biochemical properties, are essential components mammalian cellular membranes. Their importance is emphasized by the consequences defects in plasmalogen biosynthesis, which humans cause fatal disease rhizomelic chondrodysplasia punctata (RCDP). In present lipidomic study, we used fibroblasts derived from RCDP patients, as well brain tissue plasmalogen-deficient mice, examine...
Primary cardiac angiosarcomas are rare tumors with unfavorable prognosis. Pathogenic driver mutations largely unknown. We therefore analyzed a collection of cases for genomic aberrations using SNP arrays and targeted next-generation sequencing (tNGS) oncogenes tumor-suppressor genes. Recurrent gains chromosome 1q small region 4 encompassing KDR KIT were identified by array analysis. Repeatedly mutated genes tNGS different nonsynonymous mutations, MLL2 nonsense PLCG1 recurrent mutation...