000269527 001__ 269527 000269527 005__ 20240926143148.0 000269527 0247_ $$2pmc$$apmc:PMC11091963 000269527 0247_ $$2doi$$a10.1021/acsptsci.4c00037 000269527 0247_ $$2altmetric$$aaltmetric:162940424 000269527 0247_ $$2pmid$$apmid:38751620 000269527 037__ $$aDZNE-2024-00568 000269527 082__ $$a610 000269527 1001_ $$aShojaei, Monireh$$b0 000269527 245__ $$aDevelopment and Preclinical Evaluation of a Copper-64-Labeled Antibody Targeting Glycine-Alanine Dipeptides for PET Imaging of C9orf72-Associated Amyotrophic Lateral Sclerosis/Frontotemporal Dementia 000269527 260__ $$aWashington, DC$$bACS Publications$$c2024 000269527 3367_ $$2DRIVER$$aarticle 000269527 3367_ $$2DataCite$$aOutput Types/Journal article 000269527 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1715586857_2123 000269527 3367_ $$2BibTeX$$aARTICLE 000269527 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000269527 3367_ $$00$$2EndNote$$aJournal Article 000269527 520__ $$aAggregating poly(glycine-alanine) (poly-GA) is derived from the unconventional translation of the pathogenic intronic hexanucleotide repeat expansion in the C9orf72 gene, which is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Poly-GA accumulates predominantly in neuronal cytoplasmic inclusions unique to C9orf72 ALS/FTD patients. Poly-GA is, therefore, a promising target for PET/CT imaging of FTD/ALS to monitor disease progression and therapeutic interventions. A novel 64Cu-labeled anti-GA antibody (mAb1A12) targeting the poly-GA protein was developed and evaluated in a transgenic mouse model. It was obtained with high radiochemical purity (RCP), radiochemical yield (RCY), and specific activity, and showed high stability in vitro and ex vivo and specifically bound to poly-GA. The affinity of NODAGA-mAb1A12 for poly-GA was not affected by this modification. [64Cu]Cu-NODAGA-mAb1A12 was injected into transgenic mice expressing GFP-(GA)175 in excitatory neurons driven by Camk2a-Cre and in control littermates. PET/CT imaging was performed at 2, 20, and 40 h post-injection (p.i.) and revealed a higher accumulation in the cortex in transgenic mice than in wild-type mice, as reflected by higher standardized uptake value ratios (SUVR) using the cerebellum as the reference region. The organs were isolated for biodistribution and ex vivo autoradiography. Autoradiography revealed a higher cortex-to-cerebellum ratio in the transgenic mice than in the controls. Results from autoradiography were validated by immunohistochemistry and poly-GA immunoassays. Moreover, we confirmed antibody uptake in the CNS in a pharmacokinetic study of the perfused tissues. In summary, [64Cu]Cu-NODAGA-mAb1A12 demonstrated favorable in vitro characteristics and an increased relative binding in poly-GA transgenic mice compared to wild-type mice in vivo. Our results with this first-in-class radiotracer suggested that targeting poly-GA is a promising approach for PET/CT imaging in FTD/ALS. 000269527 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0 000269527 588__ $$aDataset connected to CrossRef, Journals: pub.dzne.de 000269527 7001_ $$0P:(DE-2719)2811347$$aZhou, Qihui$$b1$$udzne 000269527 7001_ $$aPalumbo, Giovanna$$b2 000269527 7001_ $$aSchaefer, Rebecca$$b3 000269527 7001_ $$aKaskinoro, Janne$$b4 000269527 7001_ $$aVehmaan-Kreula, Pirjo$$b5 000269527 7001_ $$aBartenstein, Peter$$b6 000269527 7001_ $$0P:(DE-2719)9001539$$aBrendel, Matthias$$b7$$udzne 000269527 7001_ $$0P:(DE-2719)2231621$$aEdbauer, Dieter$$b8$$udzne 000269527 7001_ $$00009-0007-4379-4436$$aLindner, Simon$$b9 000269527 773__ $$0PERI:(DE-600)2934670-8$$a10.1021/acsptsci.4c00037$$gVol. 7, no. 5, p. 1404 - 1414$$n5$$p1404 - 1414$$tACS pharmacology & translational science$$v7$$x2575-9108$$y2024 000269527 8564_ $$uhttps://pub.dzne.de/record/269527/files/DZNE-2024-00568%20SUP.pdf 000269527 8564_ $$uhttps://pub.dzne.de/record/269527/files/DZNE-2024-00568.pdf$$yOpenAccess 000269527 8564_ $$uhttps://pub.dzne.de/record/269527/files/DZNE-2024-00568%20SUP.pdf?subformat=pdfa$$xpdfa 000269527 8564_ $$uhttps://pub.dzne.de/record/269527/files/DZNE-2024-00568.pdf?subformat=pdfa$$xpdfa$$yOpenAccess 000269527 909CO $$ooai:pub.dzne.de:269527$$pdnbdelivery$$pdriver$$pVDB$$popen_access$$popenaire 000269527 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2811347$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b1$$kDZNE 000269527 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)9001539$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b7$$kDZNE 000269527 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2231621$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b8$$kDZNE 000269527 9131_ $$0G:(DE-HGF)POF4-352$$1G:(DE-HGF)POF4-350$$2G:(DE-HGF)POF4-300$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bGesundheit$$lNeurodegenerative Diseases$$vDisease Mechanisms$$x0 000269527 9141_ $$y2024 000269527 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)1190$$2StatID$$aDBCoverage$$bBiological Abstracts$$d2023-10-27 000269527 915__ $$0LIC:(DE-HGF)CCBY4$$2HGFVOC$$aCreative Commons Attribution CC BY 4.0 000269527 915__ $$0StatID:(DE-HGF)0100$$2StatID$$aJCR$$bACS PHARMACOL TRANSL : 2022$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0112$$2StatID$$aWoS$$bEmerging Sources Citation Index$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0510$$2StatID$$aOpenAccess 000269527 915__ $$0StatID:(DE-HGF)9905$$2StatID$$aIF >= 5$$bACS PHARMACOL TRANSL : 2022$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0320$$2StatID$$aDBCoverage$$bPubMed Central$$d2023-10-27 000269527 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2023-10-27 000269527 9201_ $$0I:(DE-2719)1110004$$kAG Edbauer$$lCell Biology of Neurodegeneration$$x0 000269527 9201_ $$0I:(DE-2719)1110007$$kAG Haass$$lMolecular Neurodegeneration$$x1 000269527 9201_ $$0I:(DE-2719)5000080$$kAG Zhou$$lAdaptive Immunity in Neurodegeneration$$x2 000269527 980__ $$ajournal 000269527 980__ $$aVDB 000269527 980__ $$aUNRESTRICTED 000269527 980__ $$aI:(DE-2719)1110004 000269527 980__ $$aI:(DE-2719)1110007 000269527 980__ $$aI:(DE-2719)5000080 000269527 9801_ $$aFullTexts