Klinge, Lars

[["dc.bibliographiccitation.issue","3"],["dc.bibliographiccitation.journal","European Journal of Pediatrics"],["dc.bibliographiccitation.volume","168"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Thoms, Sven"],["dc.contributor.author","Cierny, Irmgard"],["dc.contributor.author","Straub, Volker"],["dc.contributor.author","Bushby, Kate"],["dc.contributor.author","Gärtner, Jutta"],["dc.date.accessioned","2018-11-07T08:31:59Z"],["dc.date.available","2018-11-07T08:31:59Z"],["dc.date.issued","2009"],["dc.identifier.isi","000262826600054"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/17244"],["dc.language.iso","en"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Springer"],["dc.publisher.place","New york"],["dc.title","Defective membrane tubulation in dysferlin-deficient muscular dystrophy"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","6278"],["dc.bibliographiccitation.issue","22"],["dc.bibliographiccitation.journal","Human Molecular Genetics"],["dc.bibliographiccitation.lastpage","6292"],["dc.bibliographiccitation.volume","24"],["dc.contributor.author","Yuen, Michaela"],["dc.contributor.author","Cooper, Sandra T."],["dc.contributor.author","Marston, Steven"],["dc.contributor.author","Nowak, Kristen J."],["dc.contributor.author","McNamara, Elyshia"],["dc.contributor.author","Mokbel, Nancy"],["dc.contributor.author","Ilkovski, Biljana"],["dc.contributor.author","Ravenscroft, Gianina"],["dc.contributor.author","Rendu, John"],["dc.contributor.author","de Winter, Josine M."],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Beggs, Alan H."],["dc.contributor.author","North, Kathryn N."],["dc.contributor.author","Ottenheijm, Coen A. C."],["dc.contributor.author","Clarke, Nigel F."],["dc.date.accessioned","2018-11-07T09:48:54Z"],["dc.date.available","2018-11-07T09:48:54Z"],["dc.date.issued","2015"],["dc.description.abstract","Dominant mutations in TPM3, encoding alpha-tropomyosin(slow), cause a congenital myopathy characterized by generalized muscle weakness. Here, we used a multidisciplinary approach to investigate the mechanism of muscle dysfunction in 12 TPM3myopathy patients. We confirm that slow myofibre hypotrophy is a diagnostic hallmark of TPM3-myopathy, and is commonly accompanied by skewing of fibre-type ratios (either slow or fast fibre predominance). Patient muscle contained normal ratios of the three tropomyosin isoforms and normal fibre-type expression of myosins and troponins. Using 2D-PAGE, we demonstrate that mutant alpha-tropomyosin(slow) was expressed, suggesting muscle dysfunction is due to a dominant-negative effect of mutant protein on muscle contraction. Molecular modelling suggested mutant alpha-tropomyosin(slow) likely impacts actin-tropomyosin interactions and, indeed, co-sedimentation assays showed reduced binding of mutant alpha-tropomyosin(slow) (R168C) to filamentous actin. Single fibre contractility studies of patient myofibres revealed marked slow myofibre specific abnormalities. At saturating [Ca2+] (pCa 4.5), patient slow fibres produced only 63% of the contractile force produced in control slow fibres and had reduced acto-myosin cross-bridge cycling kinetics. Importantly, due to reduced Ca2+-sensitivity, at sub-saturating [Ca2+] (pCa 6, levels typically released during in vivo contraction) patient slow fibres produced only 26% of the force generated by control slow fibres. Thus, weakness in TPM3-myopathy patients can be directly attributed to reduced slow fibre force at physiological [Ca2+], and impaired acto-myosin cross-bridge cycling kinetics. Fastmyofibres are spared; however, they appear to be unable to compensate for slow fibre dysfunction. Abnormal Ca2+-sensitivity in TPM3-myopathy patients suggests Ca2+-sensitizing drugs may represent a useful treatment for this condition."],["dc.identifier.doi","10.1093/hmg/ddv334"],["dc.identifier.isi","000368371000002"],["dc.identifier.pmid","26307083"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/35400"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Oxford Univ Press"],["dc.relation.issn","1460-2083"],["dc.relation.issn","0964-6906"],["dc.title","Muscle weakness in TPM3-myopathy is due to reduced Ca2+-sensitivity and impaired acto-myosin cross-bridge cycling in slow fibres"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","946"],["dc.bibliographiccitation.issue","9"],["dc.bibliographiccitation.journal","Journal of Neurology Neurosurgery & Psychiatry"],["dc.bibliographiccitation.lastpage","953"],["dc.bibliographiccitation.volume","81"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Aboumousa, Ahmed"],["dc.contributor.author","Eagle, Michelle"],["dc.contributor.author","Hudson, Judith"],["dc.contributor.author","Sarkozy, Anna"],["dc.contributor.author","Vita, Gianluca"],["dc.contributor.author","Charlton, Richard"],["dc.contributor.author","Roberts, Mark"],["dc.contributor.author","Straub, Volker"],["dc.contributor.author","Barresi, Rita"],["dc.contributor.author","Lochmueller, Hanns"],["dc.contributor.author","Bushby, Kate"],["dc.date.accessioned","2018-11-07T08:39:53Z"],["dc.date.available","2018-11-07T08:39:53Z"],["dc.date.issued","2010"],["dc.description.abstract","Mutations in the dysferlin gene lead to limb girdle muscular dystrophy 2B, Miyoshi myopathy and distal anterior compartment myopathy. A cohort of 36 patients affected by dysferlinopathy is described, in the first UK study of clinical, genetic, pathological and biochemical data. The diagnosis was established by reduction of dysferlin in the muscle biopsy and subsequent mutational analysis of the dysferlin gene. Seventeen mutations were novel; the majority of mutations were small deletions/insertions, and no mutational hotspots were identified. Sixty-one per cent of patients ( 22 patients) initially presented with limb girdle muscular dystrophy 2B, 31% ( 11 patients) with a Miyoshi phenotype, one patient with proximodistal mode of onset, one patient with muscle stiffness after exercise and one patient as a symptomatic carrier. A wider range of age of onset was noted than previously reported, with 25% of patients having first symptoms before the age of 13 years. Independent of the initial mode of presentation, in our cohort of patients the gastrocnemius muscle was the most severely affected muscle leading to an inability to stand on tiptoes, and lower limbs were affected more severely than upper limbs. As previous anecdotal evidence on patients affected by dysferlinopathy suggests good muscle prowess before onset of symptoms, we also investigated pre-symptomatic fitness levels of the patients. Fifty-three per cent of the patients were very active and sporty before the onset of symptoms which makes the clinical course of dysferlinopathy unusual within the different forms of muscular dystrophy and provides a challenge to understanding the underlying pathomechanisms in this disease."],["dc.identifier.doi","10.1136/jnnp.2009.178038"],["dc.identifier.isi","000281313200004"],["dc.identifier.pmid","19528035"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/19107"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","B M J Publishing Group"],["dc.relation.issn","0022-3050"],["dc.title","New aspects on patients affected by dysferlin deficient muscular dystrophy"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","1119"],["dc.bibliographiccitation.issue","7"],["dc.bibliographiccitation.journal","Europace"],["dc.bibliographiccitation.lastpage","1131"],["dc.bibliographiccitation.volume","22"],["dc.contributor.author","Hofhuis, Julia"],["dc.contributor.author","Bersch, Kristina"],["dc.contributor.author","Wagner, Stefan"],["dc.contributor.author","Molina, Cristina Espinosa"],["dc.contributor.author","Fakuade, Funsho E."],["dc.contributor.author","Iyer, Lavanya M."],["dc.contributor.author","Streckfuß-Bömeke, Katrin"],["dc.contributor.author","Toischer, Karl"],["dc.contributor.author","Zelarayan, Laura Cecilia"],["dc.contributor.author","Voigt, Niels"],["dc.contributor.author","Nikolaev, Viacheslav O."],["dc.contributor.author","Maier, Lars Siegfried"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Thoms, Sven"],["dc.date.accessioned","2020-08-10T05:34:04Z"],["dc.date.available","2020-08-10T05:34:04Z"],["dc.date.issued","2020"],["dc.description.abstract","The multi-C2 domain protein dysferlin localizes to the T-Tubule system of skeletal and heart muscles. In skeletal muscle, dysferlin is known to play a role in membrane repair and in T-tubule biogenesis and maintenance. Dysferlin deficiency manifests as muscular dystrophy of proximal and distal muscles. Cardiomyopathies have been also reported, and some dysferlinopathy mouse models develop cardiac dysfunction under stress. Generally, the role and functional relevance of dysferlin in the heart is not clear. The aim of this study was to analyse the effect of dysferlin deficiency on the transverse-axial tubule system (TATS) structure and on Ca2+ homeostasis in the heart."],["dc.identifier.doi","10.1093/europace/euaa093"],["dc.identifier.pmid","32572487"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/67547"],["dc.identifier.url","https://sfb1002.med.uni-goettingen.de/production/literature/publications/360"],["dc.language.iso","en"],["dc.relation","SFB 1002: Modulatorische Einheiten bei Herzinsuffizienz"],["dc.relation","SFB 1002 | A10: Peroxisomen als modulatorische Einheiten im Herzstoffwechsel und bei Herzinsuffizienz"],["dc.relation","SFB 1002 | C07: Kardiomyozyten Wnt/β-catenin Komplex Aktivität im pathologischen Herz-Remodeling - als gewebespezifischer therapeutischer Ansatz"],["dc.relation","SFB 1002 | D04: Bedeutung der Methylierung von RNA (m6A) und des Histons H3 (H3K4) in der Herzinsuffizienz"],["dc.relation","SFB 1002 | S01: In vivo und in vitro Krankheitsmodelle"],["dc.relation","SFB 1002 | A13: Bedeutung einer gestörten zytosolischen Calciumpufferung bei der atrialen Arrhythmogenese bei Patienten mit Herzinsuffizienz (HF)"],["dc.relation.eissn","1532-2092"],["dc.relation.issn","1099-5129"],["dc.relation.workinggroup","RG L. Maier (Experimentelle Kardiologie)"],["dc.relation.workinggroup","RG Nikolaev (Cardiovascular Research Center)"],["dc.relation.workinggroup","RG Thoms (Biochemistry and Molecular Medicine)"],["dc.relation.workinggroup","RG Toischer (Kardiales Remodeling)"],["dc.relation.workinggroup","RG Voigt (Molecular Pharmacology)"],["dc.relation.workinggroup","RG Zelarayán-Behrend (Developmental Pharmacology)"],["dc.title","Dysferlin links excitation-contraction coupling to structure and maintenance of the cardiac transverse-axial tubule system"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.subtype","original_ja"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","779"],["dc.bibliographiccitation.issue","7"],["dc.bibliographiccitation.journal","Human Mutation"],["dc.bibliographiccitation.lastpage","790"],["dc.bibliographiccitation.volume","35"],["dc.contributor.author","Marttila, Minttu"],["dc.contributor.author","Lehtokari, Vilma-Lotta"],["dc.contributor.author","Marston, Steven"],["dc.contributor.author","Nyman, Tuula A."],["dc.contributor.author","Barnerias, Christine"],["dc.contributor.author","Beggs, Alan H."],["dc.contributor.author","Bertini, Enrico"],["dc.contributor.author","Ceyhan-Birsoy, Oezge"],["dc.contributor.author","Cintas, Pascal"],["dc.contributor.author","Gerard, Marion"],["dc.contributor.author","Gilbert-Dussardier, Brigitte"],["dc.contributor.author","Hogue, Jacob S."],["dc.contributor.author","Longman, Cheryl"],["dc.contributor.author","Eymard, Bruno"],["dc.contributor.author","Frydman, Moshe"],["dc.contributor.author","Kang, Peter B."],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Kolski, Hanna"],["dc.contributor.author","Lochmueller, Hans"],["dc.contributor.author","Magy, Laurent"],["dc.contributor.author","Manel, Veronique"],["dc.contributor.author","Mayer, Michele"],["dc.contributor.author","Mercuri, Eugenio"],["dc.contributor.author","North, Kathryn N."],["dc.contributor.author","Peudenier-Robert, Sylviane"],["dc.contributor.author","Pihko, Helena"],["dc.contributor.author","Probst, Frank J."],["dc.contributor.author","Reisin, Ricardo"],["dc.contributor.author","Stewart, Willie"],["dc.contributor.author","Taratuto, Ana Lia"],["dc.contributor.author","de Visser, Marianne"],["dc.contributor.author","Wilichowski, Ekkehard"],["dc.contributor.author","Winer, John"],["dc.contributor.author","Nowak, Kristen"],["dc.contributor.author","Laing, Nigel G."],["dc.contributor.author","Winder, Tom L."],["dc.contributor.author","Monnier, Nicole"],["dc.contributor.author","Clarke, Nigel F."],["dc.contributor.author","Pelin, Katarina"],["dc.contributor.author","Groenholm, Mikaela"],["dc.contributor.author","Wallgren-Pettersson, Carina"],["dc.date.accessioned","2018-11-07T09:38:29Z"],["dc.date.available","2018-11-07T09:38:29Z"],["dc.date.issued","2014"],["dc.description.abstract","Mutations affecting skeletal muscle isoforms of the tropomyosin genes may cause nemaline myopathy, cap myopathy, core-rod myopathy, congenital fiber-type disproportion, distal arthrogryposes, and Escobar syndrome. We correlate the clinical picture of these diseases with novel (19) and previously reported (31) mutations of the TPM2 and TPM3 genes. Included are altogether 93 families: 53 with TPM2 mutations and 40 with TPM3 mutations. Thirty distinct pathogenic variants of TPM2 and 20 of TPM3 have been published or listed in the Leiden Open Variant Database (http://www.dmd.nl/). Most are heterozygous changes associated with autosomal-dominant disease. Patients with TPM2 mutations tended to present with milder symptoms than those with TPM3 mutations, DA being present only in the TPM2 group. Previous studies have shown that five of the mutations in TPM2 and one in TPM3 cause increased Ca2+ sensitivity resulting in a hypercontractile molecular phenotype. Patients with hypercontractile phenotype more often had contractures of the limb joints (18/19) and jaw (6/19) than those with nonhypercontractile ones (2/22 and 1/22), whereas patients with the non-hypercontractile molecular phenotype more often (19/22) had axial contractures than the hypercontractile group (7/19). Our in silico predictions show that most mutations affect tropomyosin-actin association or tropomyosin head-to-tail binding."],["dc.identifier.doi","10.1002/humu.22554"],["dc.identifier.isi","000337705500001"],["dc.identifier.pmid","24692096"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/33074"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-blackwell"],["dc.relation.issn","1098-1004"],["dc.relation.issn","1059-7794"],["dc.title","Mutation Update and Genotype-Phenotype Correlations of Novel and Previously Described Mutations in TPM2 and TPM3 Causing Congenital Myopathies"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.issue","9-10"],["dc.bibliographiccitation.journal","Neuromuscular Disorders"],["dc.bibliographiccitation.volume","24"],["dc.contributor.author","Hobbiebrunken, Elke"],["dc.contributor.author","Schulz-Schaeffer, Walter J."],["dc.contributor.author","Podskarbi, T."],["dc.contributor.author","Mueller-Reible, Clemens"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Goebel, Hans H."],["dc.contributor.author","Wilichowski, E."],["dc.date.accessioned","2018-11-07T09:34:36Z"],["dc.date.available","2018-11-07T09:34:36Z"],["dc.date.issued","2014"],["dc.format.extent","886"],["dc.identifier.doi","10.1016/j.nmd.2014.06.308"],["dc.identifier.isi","000342870200305"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/32204"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Pergamon-elsevier Science Ltd"],["dc.publisher.place","Oxford"],["dc.relation.conference","19th International Congress of the World-Muscle-Society"],["dc.relation.eventlocation","Berlin, GERMANY"],["dc.relation.issn","1873-2364"],["dc.relation.issn","0960-8966"],["dc.title","Double trouble: A child with spinal muscular atrophy type III and Pompe disease"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.issue","9-10"],["dc.bibliographiccitation.journal","Neuromuscular Disorders"],["dc.bibliographiccitation.volume","21"],["dc.contributor.author","Zschuentzsch, Jana"],["dc.contributor.author","Makosch, Gregor"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Tiburcy, Malte"],["dc.contributor.author","Brinkmeier, Heinrich"],["dc.contributor.author","Liebetan, D."],["dc.contributor.author","Schmidt, J."],["dc.date.accessioned","2018-11-07T08:51:10Z"],["dc.date.available","2018-11-07T08:51:10Z"],["dc.date.issued","2011"],["dc.format.extent","710"],["dc.identifier.doi","10.1016/j.nmd.2011.06.984"],["dc.identifier.isi","000295955900233"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/21866"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Pergamon-elsevier Science Ltd"],["dc.publisher.place","Oxford"],["dc.relation.conference","16th International Congress of the World-Muscle-Society"],["dc.relation.eventlocation","Algarve, PORTUGAL"],["dc.relation.issn","0960-8966"],["dc.title","Human IgG improves the performance of mdx mice in voluntary wheel running"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","695"],["dc.bibliographiccitation.issue","4"],["dc.bibliographiccitation.journal","Circulation Research"],["dc.bibliographiccitation.lastpage","704"],["dc.bibliographiccitation.volume","106"],["dc.contributor.author","Knöll, Ralph"],["dc.contributor.author","Kostin, Sawa"],["dc.contributor.author","Klede, Stefanie"],["dc.contributor.author","Savvatis, Kostas"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Stehle, Ina"],["dc.contributor.author","Gunkel, Sylvia"],["dc.contributor.author","Kötter, Sebastian"],["dc.contributor.author","Babicz, Kamila"],["dc.contributor.author","Sohns, Melanie"],["dc.contributor.author","Miocic, Snjezana"],["dc.contributor.author","Didie, Michael"],["dc.contributor.author","Knöll, Gudrun"],["dc.contributor.author","Zimmermann, Wolfram-Hubertus"],["dc.contributor.author","Thelen, Paul"],["dc.contributor.author","Bickeböller, Heike"],["dc.contributor.author","Maier, Lars S."],["dc.contributor.author","Schaper, Wolfgang"],["dc.contributor.author","Schaper, Jutta"],["dc.contributor.author","Kraft, Theresia"],["dc.contributor.author","Tschöpe, Carsten"],["dc.contributor.author","Linke, Wolfgang A."],["dc.contributor.author","Chien, Kenneth R."],["dc.date.accessioned","2017-09-07T11:46:07Z"],["dc.date.available","2017-09-07T11:46:07Z"],["dc.date.issued","2010"],["dc.description.abstract","Rationale: We previously discovered the human 10T -> C (Trp4Arg) missense mutation in exon 2 of the muscle LIM protein (MLP, CSRP3) gene. Objective: We sought to study the effects of this single-nucleotide polymorphism in the in vivo situation. Methods and Results: We now report the generation and detailed analysis of the corresponding Mlp(W4R/+) and Mlp(W4R/W4R) knock-in animals, which develop an age-and gene dosage-dependent hypertrophic cardiomyopathy and heart failure phenotype, characterized by almost complete loss of contractile reserve under catecholamine induced stress. In addition, evidence for skeletal muscle pathology, which might have implications for human mutation carriers, was observed. Importantly, we found significantly reduced MLP mRNA and MLP protein expression levels in hearts of heterozygous and homozygous W4R-MLP knock-in animals. We also detected a weaker in vitro interaction of telethonin with W4R-MLP than with wild-type MLP. These alterations may contribute to an increased nuclear localization of W4R-MLP, which was observed by immunohistochemistry. Conclusions: Given the well-known high frequency of this mutation in Caucasians of up to 1%, our data suggest that W4R-MLP might contribute significantly to human cardiovascular disease. (Circ Res. 2010; 106: 695-704.)"],["dc.identifier.doi","10.1161/CIRCRESAHA.109.206243"],["dc.identifier.gro","3142953"],["dc.identifier.isi","000275190500010"],["dc.identifier.pmid","20044516"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/414"],["dc.language.iso","en"],["dc.notes.intern","WoS Import 2017-03-10"],["dc.notes.status","final"],["dc.notes.submitter","PUB_WoS_Import"],["dc.relation.issn","0009-7330"],["dc.title","A Common MLP (Muscle LIM Protein) Variant Is Associated With Cardiomyopathy"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.subtype","original"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","351"],["dc.bibliographiccitation.issue","2"],["dc.bibliographiccitation.journal","Journal of Neurochemistry"],["dc.bibliographiccitation.lastpage","362"],["dc.bibliographiccitation.volume","136"],["dc.contributor.author","Zschuentzsch, Jana"],["dc.contributor.author","Zhang, Y."],["dc.contributor.author","Klinker, Florian"],["dc.contributor.author","Makosch, Gregor"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Malzahn, Doerthe"],["dc.contributor.author","Brinkmeier, Heinrich"],["dc.contributor.author","Liebetanz, David"],["dc.contributor.author","Schmidt, Jens"],["dc.date.accessioned","2018-11-07T10:21:27Z"],["dc.date.available","2018-11-07T10:21:27Z"],["dc.date.issued","2016"],["dc.description.abstract","Duchenne muscular dystrophy (DMD) is a severe hereditary myopathy. Standard treatment by glucocorticosteroids is limited because of numerous side effects. The aim of this study was to test immunomodulation by human immunoglobulin G (IgG) as treatment in the experimental mouse model (mdx) of DMD. 2g/kg human IgG compared to human albumin was injected intraperitoneally in mdx mice at the age of 3 and 7weeks. Advanced voluntary wheel running parameters were recorded continuously. At the age of 11 weeks, animals were killed so that blood, diaphragm, and lower limb muscles could be removed for quantitative PCR, histological analysis and exvivo muscle contraction tests. IgG compared to albumin significantly improved the voluntary running performance and reduced muscle fatigability in an exvivo muscle contraction test. Upon IgG treatment, serum creatine kinase values were diminished and mRNA expression levels of relevant inflammatory markers were reduced in the diaphragm and limb muscles. Macrophage infiltration and myopathic damage were significantly ameliorated in the quadriceps muscle. Collectively, this study demonstrates that, in the early disease course of mdx mice, human IgG improves the running performance and diminishes myopathic damage and inflammation in the muscle. Therefore, IgG may be a promising approach for treatment of DMD."],["dc.identifier.doi","10.1111/jnc.13269"],["dc.identifier.isi","000367956800015"],["dc.identifier.pmid","26230042"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/42093"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Wiley-blackwell"],["dc.relation.issn","1471-4159"],["dc.relation.issn","0022-3042"],["dc.title","Treatment with human immunoglobulin G improves the early disease course in a mouse model of Duchenne muscular dystrophy"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","177"],["dc.bibliographiccitation.issue","1-2"],["dc.bibliographiccitation.journal","Journal of Neuroimmunology"],["dc.bibliographiccitation.lastpage","178"],["dc.bibliographiccitation.volume","253"],["dc.contributor.author","Zschuentzsch, Jana"],["dc.contributor.author","Weller, Charlotte"],["dc.contributor.author","Zhang, Y."],["dc.contributor.author","Klinker, Florian"],["dc.contributor.author","Makosch, Gregor"],["dc.contributor.author","Klinge, Lars"],["dc.contributor.author","Metselaar, Josbert M."],["dc.contributor.author","Liebetanz, David"],["dc.contributor.author","Brinkmeier, Heinrich"],["dc.contributor.author","Schmidt, Jens"],["dc.date.accessioned","2018-11-07T09:02:18Z"],["dc.date.available","2018-11-07T09:02:18Z"],["dc.date.issued","2012"],["dc.identifier.isi","000312764800478"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/24650"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Elsevier Science Bv"],["dc.publisher.place","Amsterdam"],["dc.relation.conference","11th International Congress of Neuroimmunology (ISNI)"],["dc.relation.eventlocation","Boston, MA"],["dc.relation.issn","0165-5728"],["dc.title","Human IgG but not liposomal prednisolone improves the early clinical course of mdx mice as model for Duchenne muscular dystrophy"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]