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Akademický článek

2-Year-Old and 3-Year-Old Italian ALS Patients with Novel ALS2 Mutations: Identification of Key Metabolites in Their Serum and Plasma

Autor: Mukesh Gautam, Renata Del Carratore, Benjamin Helmold, Alessandra Tessa, Oge Gozutok, Navdeep Chandel, Halil Idrisoglu, Paolo Bongioanni, Roberta Battini, P.Hande Ozdinler

Publikováno v: Metabolites, Vol 12, Iss 2, p 174 (2022)

Pathogenic variants in ALS2 have been detected mostly in juvenile cases of amyotrophic lateral sclerosis (ALS), affecting mainly children and teenagers. Patients with ALS2 mutations demonstrate early onset cortical involvement in ALS. Currently, ther

Externí odkaz: https://doaj.org/article/9dd60907849b4ab3b3bd9c5fb1d6b964

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Akademický článek

Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS

Autor: Oge Gozutok, Benjamin Ryan Helmold, P. Hande Ozdinler

Publikováno v: Brain Sciences, Vol 11, Iss 5, p 578 (2021)

Hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS) are rare motor neuron diseases, which affect mostly the upper motor neurons (UMNs) in patients. The UMNs display early vulnerability and progressive degeneration, while other cor

Externí odkaz: https://doaj.org/article/1adf1f94e64647c98c30ceb5f3da2b6c

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Akademický článek

The Timing and Extent of Motor Neuron Vulnerability in ALS Correlates with Accumulation of Misfolded SOD1 Protein in the Cortex and in the Spinal Cord

Autor: Baris Genc, Oge Gozutok, Nuran Kocak, P. Hande Ozdinler

Publikováno v: Cells, Vol 9, Iss 2, p 502 (2020)

Understanding the cellular and molecular basis of selective vulnerability has been challenging, especially for motor neuron diseases. Developing drugs that improve the health of neurons that display selective vulnerability relies on in vivo cell-base

Externí odkaz: https://doaj.org/article/2fff28d4bc034e88985efb9a06fb6151

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2-Year-Old and 3-Year-Old Italian ALS Patients with Novel

Autor: Mukesh, Gautam, Renata Del, Carratore, Benjamin, Helmold, Alessandra, Tessa, Oge, Gozutok, Navdeep, Chandel, Halil, Idrisoglu, Paolo, Bongioanni, Roberta, Battini, P Hande, Ozdinler

Publikováno v: Metabolites. 12(2)

Pathogenic variants in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::4978b715c74dd7834f2b3f98efdc864b
https://pubmed.ncbi.nlm.nih.gov/35208248

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Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons

Autor: Yongling Zhu, Oge Gozutok, Javier H. Jara, Nuran Kocak, Santana S Sanchez, Barış Genç, P. Hande Özdinler, Amiko K. B. Lagrimas

Publikováno v: Gene therapy. 29(3-4)

There are no effective cures for upper motor neuron (UMN) diseases, such as amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, and hereditary spastic paraplegia. Here, we show UMN loss occurs independent of spinal motor neuron degenerati

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::906ce851ab9ae2c12433891bd6859a8d
https://pubmed.ncbi.nlm.nih.gov/34853443

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Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS

Autor: P. Hande Özdinler, Benjamin Ryan Helmold, Oge Gozutok

Publikováno v: Brain Sciences, Vol 11, Iss 578, p 578 (2021)
Brain Sciences
Brain Sciences; Volume 11; Issue 5; Pages: 578

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cf7ef50746d57acacdf68451c9260801
https://www.mdpi.com/2076-3425/11/5/578

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Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP‐43 pathology

Autor: Ina Dervishi, Santana S Sanchez, Edward F. Xie, Gashaw M. Goshu, P. Hande Özdinler, Mukesh Gautam, Oge Gozutok, Nuran Kocak, Barış Genç, Richard B. Silverman

Publikováno v: Clinical and Translational Medicine, Vol 11, Iss 2, Pp n/a-n/a (2021)
Clinical and Translational Medicine

Background Upper motor neurons (UMNs) are a key component of motor neuron circuitry. Their degeneration is a hallmark for diseases, such as hereditary spastic paraplegia (HSP), primary lateral sclerosis (PLS), and amyotrophic lateral sclerosis (ALS).

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::18097f37676ed2d9c74b4ab19937e11d
https://doaj.org/article/5370d3704c9349de99b927f4e9b91ea3

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Better understanding the neurobiology of primary lateral sclerosis

Autor: Georg Haase, Csaba Konrad, Marcella L. Erb, Oge Gozutok, Estela Area Gomez, Giovanni Manfredi, Zheng Tian, P. Hande Özdinler, Mukesh Gautam, Hiroshi Mitsumoto

Publikováno v: Amyotroph Lateral Scler Frontotemporal Degener

Primary lateral sclerosis (PLS) is a rare neurodegenerative disease characterized by progressive degeneration of upper motor neurons (UMNs). Recent studies shed new light onto the cellular events that are particularly important for UMN maintenance in

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c5185db12ed6e48548f60874d5f4c9a9
https://europepmc.org/articles/PMC8016556/

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Importance of lipids for upper motor neuron health and disease

Autor: Heather H. Shin, P. Hande Özdinler, Mukesh Gautam, Aksu Gunay, Oge Gozutok

Publikováno v: Semin Cell Dev Biol

Building evidence reveals the importance of maintaining lipid homeostasis for the health and function of neurons, and the upper motor neurons (UMNs) are no exception. UMNs are critically important for the initiation and modulation of voluntary moveme

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::93f7e013ff9b9bba24ced4262fd5c6f2
https://pubmed.ncbi.nlm.nih.gov/33323321

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