Dataset in support of the generation of Niemann-Pick disease Type C1 patient-specific iPS cell lines carrying the novel NPC1 mutation c.1180T>C or the prevalent c.3182T>C mutation – Analysis of pluripotency and neuronal differentiation

Autor: Franziska Peter, Michaela Trilck, Michael Rabenstein, Arndt Rolfs, Moritz J. Frech

Publikováno v: Data in Brief, Vol 12, Iss C, Pp 123-131 (2017)

Data presented in this article demonstrate the generation and characterization of two novel Niemann-Pick disease Type C1 (NPC1) patient-specific induced pluripotent stem cell (iPSC) lines, related to the research article Trilck et al. (Diversity of G

Externí odkaz: https://doaj.org/article/159ce8fffd284fee8029a6c3a3e5cf44

Pathophysiological In Vitro Profile of Neuronal Differentiated Cells Derived from Niemann-Pick Disease Type C2 Patient-Specific iPSCs Carrying the

Autor: Maik Liedtke, Christin Völkner, Alexandra V. Jürs, Franziska Peter, Michael Rabenstein, Andreas Hermann, Moritz J. Frech

Publikováno v: International journal of molecular sciences 22(8), 4009-(2021). doi:10.3390/ijms22084009
International Journal of Molecular Sciences
International Journal of Molecular Sciences, Vol 22, Iss 4009, p 4009 (2021)

Niemann-Pick type C2 (NP-C2) disease is a rare hereditary disease caused by mutations in the NPC2 gene. NPC2 is a small, soluble protein consisting of 151 amino acids, primarily expressed in late endosomes and lysosomes (LE/LY). Together with NPC1, a

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::6f5f471425807d531953b0aa40da9458
https://pubmed.ncbi.nlm.nih.gov/33924575

Cyclodextrin Alters GABAergic Input to CA1 Pyramidal Cells in Wild-Type But Not in NPC1-Deficient Mice

Autor: Michael Rabenstein, Sebastian Rost, Arndt Rolfs, Katja Bovensiepen, Moritz J. Frech

Publikováno v: BioResearch Open Access, Vol 4, Iss 1, Pp 358-362 (2015)
BioResearch Open Access

Niemann–Pick type C1 disease (NPC1) is a neurodegenerative disorder caused by mutations in the NPC1 gene. Actual, no causative treatment for NPC1 is available, although some drugs have been proven to be beneficial to patients, for example, 2-hydrox

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::143fa182a819abeb45b48f9f03ff1803
https://doi.org/10.1089/biores.2015.0023

Macrocycles 14. Polycondensations of Aspirin and Other Monomers of Salicylic Acid

Autor: Hans R. Kricheldorf, Tang Liming, Michael Rabenstein

Publikováno v: Macromolecular Chemistry and Physics. 202:1497-1504

Aspirin was polycondensed at various temperature-time combinations with or without the addition of a catalyst. Furthermore, several polycondensations of both the trimethylsilyl ester of aspirin, and o-trimethylsiloxybenzoyl chloride were conducted. V

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::91595fc4702e97bf986e8c26184ed30d
https://doi.org/10.1002/1521-3935(20010601)202:9<1497::aid-macp1497>3.0.co

LC-polyimide 38. Smectic poly(ester-imide)s of N,N-bis(4-hydroxy-phenyl)biphenyl-3,3′,4,4′-tetracarboxylic imide having long aliphatic spacers. Part I

Autor: Hans R. Kricheldorf, Gert Schwarz, Michael Rabenstein

Publikováno v: Polymer. 42:1789-1796

A new monomer ( 7 ) was prepared by alkylation of N , N ,-bis(4-hydroxyphenyl)biphenyl-3,3′,4,4′-tetracarboxylic imide with 11-bromoundecanol. This imide diol ( 7 ) did not form a LC-phase, but showed two reversible first-order transitions in the

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::847c08cfc90e6e944d74165a075aa62b
https://doi.org/10.1016/s0032-3861(00)00455-9