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Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease.

Frontiers in neuroanatomy

Jara JH, Genç B, Klessner JL, Ozdinler PH.
PMID: 24723858
Front Neuroanat. 2014 Mar 26;8:16. doi: 10.3389/fnana.2014.00016. eCollection 2014.

Corticospinal motor neurons (CSMN) have a unique ability to receive, integrate, translate, and transmit the cerebral cortex's input toward spinal cord targets and therefore act as a "spokesperson" for the initiation and modulation of voluntary movements that require cortical...

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Jara JH, Genç B, Klessner JL, et al. Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease. Front Neuroanat. 2014;8:16doi: 10.3389/fnana.2014.00016.
Jara, J. H., Genç, B., Klessner, J. L., & Ozdinler, P. H. (2014). Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease. Frontiers in neuroanatomy, 816. https://doi.org/10.3389/fnana.2014.00016
Jara, Javier H, et al. "Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease." Frontiers in neuroanatomy vol. 8 (2014): 16. doi: https://doi.org/10.3389/fnana.2014.00016
Jara JH, Genç B, Klessner JL, Ozdinler PH. Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease. Front Neuroanat. 2014 Mar 26;8:16. doi: 10.3389/fnana.2014.00016. eCollection 2014. PMID: 24723858; PMCID: PMC3972458.
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Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS.

Frontiers in cellular neuroscience

Gautam M, Xie EF, Kocak N, Ozdinler PH.
PMID: 31787882
Front Cell Neurosci. 2019 Nov 07;13:489. doi: 10.3389/fncel.2019.00489. eCollection 2019.

Mitochondrial dysfunction is one of the converging paths for many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), and TDP-43 pathology is the most common proteinopathy detected in ALS and ALS/Frontotemporal lobar degeneration (ALS/FTLD). We recently identified mitochondrial problems in...

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Gautam M, Xie EF, Kocak N, et al. Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS. Front Cell Neurosci. 2019;13:489doi: 10.3389/fncel.2019.00489.
Gautam, M., Xie, E. F., Kocak, N., & Ozdinler, P. H. (2019). Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS. Frontiers in cellular neuroscience, 13489. https://doi.org/10.3389/fncel.2019.00489
Gautam, Mukesh, et al. "Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS." Frontiers in cellular neuroscience vol. 13 (2019): 489. doi: https://doi.org/10.3389/fncel.2019.00489
Gautam M, Xie EF, Kocak N, Ozdinler PH. Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS. Front Cell Neurosci. 2019 Nov 07;13:489. doi: 10.3389/fncel.2019.00489. eCollection 2019. PMID: 31787882; PMCID: PMC6854036.
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Importance of lipids for upper motor neuron health and disease.

Seminars in cell & developmental biology

Gunay A, Shin HH, Gozutok O, Gautam M, Ozdinler PH.
PMID: 33323321
Semin Cell Dev Biol. 2021 Apr;112:92-104. doi: 10.1016/j.semcdb.2020.11.004. Epub 2020 Dec 13.

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

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Gunay A, Shin HH, Gozutok O, et al. Importance of lipids for upper motor neuron health and disease. Semin Cell Dev Biol. 2020;112:92-104doi: 10.1016/j.semcdb.2020.11.004.
Gunay, A., Shin, H. H., Gozutok, O., Gautam, M., & Ozdinler, P. H. (2021). Importance of lipids for upper motor neuron health and disease. Seminars in cell & developmental biology, 11292-104. https://doi.org/10.1016/j.semcdb.2020.11.004
Gunay, Aksu, et al. "Importance of lipids for upper motor neuron health and disease." Seminars in cell & developmental biology vol. 112 (2021): 92-104. doi: https://doi.org/10.1016/j.semcdb.2020.11.004
Gunay A, Shin HH, Gozutok O, Gautam M, Ozdinler PH. Importance of lipids for upper motor neuron health and disease. Semin Cell Dev Biol. 2021 Apr;112:92-104. doi: 10.1016/j.semcdb.2020.11.004. Epub 2020 Dec 13. PMID: 33323321; PMCID: PMC8026509.
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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.

Clinical and translational medicine

Genç B, Gautam M, Gözütok Ö, Dervishi I, Sanchez S, Goshu GM, Koçak N, Xie E, Silverman RB, Özdinler PH.
PMID: 33634973
Clin Transl Med. 2021 Feb;11(2):e336. doi: 10.1002/ctm2.336.

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). Currently there are no...

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Genç B, Gautam M, Gözütok Ö, et al. Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology. Clin Transl Med. 2021;11(2):e336doi: 10.1002/ctm2.336.
Genç, B., Gautam, M., Gözütok, �. �., Dervishi, I., Sanchez, S., Goshu, G. M., Koçak, N., Xie, E., Silverman, R. B., & Özdinler, P. H. (2021). Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology. Clinical and translational medicine, 11(2), e336. https://doi.org/10.1002/ctm2.336
Genç, Barış, et al. "Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology." Clinical and translational medicine vol. 11,2 (2021): e336. doi: https://doi.org/10.1002/ctm2.336
Genç B, Gautam M, Gözütok Ö, Dervishi I, Sanchez S, Goshu GM, Koçak N, Xie E, Silverman RB, Özdinler PH. Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology. Clin Transl Med. 2021 Feb;11(2):e336. doi: 10.1002/ctm2.336. PMID: 33634973; PMCID: PMC7898037.
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Absence of UCHL 1 function leads to selective motor neuropathy.

Annals of clinical and translational neurology

Genç B, Jara JH, Schultz MC, Manuel M, Stanford MJ, Gautam M, Klessner JL, Sekerkova G, Heller DB, Cox GA, Heckman CJ, DiDonato CJ, Özdinler PH.
PMID: 27231703
Ann Clin Transl Neurol. 2016 Mar 07;3(5):331-45. doi: 10.1002/acn3.298. eCollection 2016 May.

OBJECTIVE: The aim of this study was to investigate the role of ubiquitin C-terminal hydrolase-L1 (UCHL1) for motor neuron circuitry and especially in spinal motor neuron (SMN) health, function, and connectivity.METHODS: Since mutations in UCHL1 gene leads to motor...

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Genç B, Jara JH, Schultz MC, et al. Absence of UCHL 1 function leads to selective motor neuropathy. Ann Clin Transl Neurol. 2016;3(5):331-45doi: 10.1002/acn3.298.
Genç, B., Jara, J. H., Schultz, M. C., Manuel, M., Stanford, M. J., Gautam, M., Klessner, J. L., Sekerkova, G., Heller, D. B., Cox, G. A., Heckman, C. J., DiDonato, C. J., & Özdinler, P. H. (2016). Absence of UCHL 1 function leads to selective motor neuropathy. Annals of clinical and translational neurology, 3(5), 331-45. https://doi.org/10.1002/acn3.298
Genç, Barış, et al. "Absence of UCHL 1 function leads to selective motor neuropathy." Annals of clinical and translational neurology vol. 3,5 (2016): 331-45. doi: https://doi.org/10.1002/acn3.298
Genç B, Jara JH, Schultz MC, Manuel M, Stanford MJ, Gautam M, Klessner JL, Sekerkova G, Heller DB, Cox GA, Heckman CJ, DiDonato CJ, Özdinler PH. Absence of UCHL 1 function leads to selective motor neuropathy. Ann Clin Transl Neurol. 2016 Mar 07;3(5):331-45. doi: 10.1002/acn3.298. eCollection 2016 May. PMID: 27231703; PMCID: PMC4863746.
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The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS.

Frontiers in molecular neuroscience

Jara JH, Sheets PL, Nigro MJ, Perić M, Brooks C, Heller DB, Martina M, Andjus PR, Ozdinler PH.
PMID: 32508590
Front Mol Neurosci. 2020 May 19;13:73. doi: 10.3389/fnmol.2020.00073. eCollection 2020.

The brain is complex and heterogeneous. Even though numerous independent studies indicate cortical hyperexcitability as a potential contributor to amyotrophic lateral sclerosis (ALS) pathology, the mechanisms that are responsible for upper motor neuron (UMN) vulnerability remain elusive. To reveal...

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Jara JH, Sheets PL, Nigro MJ, et al. The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS. Front Mol Neurosci. 2020;13:73doi: 10.3389/fnmol.2020.00073.
Jara, J. H., Sheets, P. L., Nigro, M. J., Perić, M., Brooks, C., Heller, D. B., Martina, M., Andjus, P. R., & Ozdinler, P. H. (2020). The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS. Frontiers in molecular neuroscience, 1373. https://doi.org/10.3389/fnmol.2020.00073
Jara, Javier H, et al. "The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS." Frontiers in molecular neuroscience vol. 13 (2020): 73. doi: https://doi.org/10.3389/fnmol.2020.00073
Jara JH, Sheets PL, Nigro MJ, Perić M, Brooks C, Heller DB, Martina M, Andjus PR, Ozdinler PH. The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS. Front Mol Neurosci. 2020 May 19;13:73. doi: 10.3389/fnmol.2020.00073. eCollection 2020. PMID: 32508590; PMCID: PMC7248374.
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Treatment of amyotrophic lateral sclerosis: lessons learned from many failures.

ACS medicinal chemistry letters

Ozdinler PH, Silverman RB.
PMID: 25408825
ACS Med Chem Lett. 2014 Oct 08;5(11):1179-81. doi: 10.1021/ml500404b. eCollection 2014 Nov 13.

Amyotrophic lateral sclerosis (ALS) is one of the most complex neurodegenerative diseases, involving both cortical and spinal components of motor neuron circuitry and non-neuronal cells that support the motor neurons. There is no effective therapeutic for ALS, and compounds...

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Ozdinler PH, Silverman RB. Treatment of amyotrophic lateral sclerosis: lessons learned from many failures. ACS Med Chem Lett. 2014;5(11):1179-81doi: 10.1021/ml500404b.
Ozdinler, P. H., & Silverman, R. B. (2014). Treatment of amyotrophic lateral sclerosis: lessons learned from many failures. ACS medicinal chemistry letters, 5(11), 1179-81. https://doi.org/10.1021/ml500404b
Ozdinler, P Hande, and Silverman, Richard B. "Treatment of amyotrophic lateral sclerosis: lessons learned from many failures." ACS medicinal chemistry letters vol. 5,11 (2014): 1179-81. doi: https://doi.org/10.1021/ml500404b
Ozdinler PH, Silverman RB. Treatment of amyotrophic lateral sclerosis: lessons learned from many failures. ACS Med Chem Lett. 2014 Oct 08;5(11):1179-81. doi: 10.1021/ml500404b. eCollection 2014 Nov 13. PMID: 25408825; PMCID: PMC4233364.
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Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS.

Brain sciences

Gozutok O, Helmold BR, Ozdinler PH.
PMID: 33947096
Brain Sci. 2021 Apr 29;11(5). doi: 10.3390/brainsci11050578.

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 cortical neurons mostly remain functional....

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Gozutok O, Helmold BR, Ozdinler PH. Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS. Brain Sci. 2021;11(5)doi: 10.3390/brainsci11050578.
Gozutok, O., Helmold, B. R., & Ozdinler, P. H. (2021). Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS. Brain sciences, 11(5), . https://doi.org/10.3390/brainsci11050578
Gozutok, Oge, et al. "Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS." Brain sciences vol. 11,5 (2021). doi: https://doi.org/10.3390/brainsci11050578
Gozutok O, Helmold BR, Ozdinler PH. Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS. Brain Sci. 2021 Apr 29;11(5). doi: 10.3390/brainsci11050578. PMID: 33947096; PMCID: PMC8146506.
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Deciphering the molecular logic of ALS using model organisms: "A family affair.".

SOJ neurology

Brent JR, Ozdinler PH.
PMID: 33693055
SOJ Neurol. 2017;4(1):1-3. doi: 10.15226/2374-6858/4/1/00132. Epub 2017 Jul 19.

Recent advances in the genetics of ALS have bolstered hope that a molecular logic for the pathogenesis of the disease is fast approaching. An emerging challenge is the dissection of the common and unique molecular pathways altered by ALS...

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Brent JR, Ozdinler PH. Deciphering the molecular logic of ALS using model organisms: "A family affair.". SOJ Neurol. 2017;4(1):1-3doi: 10.15226/2374-6858/4/1/00132.
Brent, J. R., & Ozdinler, P. H. (2017). Deciphering the molecular logic of ALS using model organisms: "A family affair.". SOJ neurology, 4(1), 1-3. https://doi.org/10.15226/2374-6858/4/1/00132
Brent, Jonathan R, and Ozdinler, P Hande. "Deciphering the molecular logic of ALS using model organisms: "A family affair."." SOJ neurology vol. 4,1 (2017): 1-3. doi: https://doi.org/10.15226/2374-6858/4/1/00132
Brent JR, Ozdinler PH. Deciphering the molecular logic of ALS using model organisms: "A family affair.". SOJ Neurol. 2017;4(1):1-3. doi: 10.15226/2374-6858/4/1/00132. Epub 2017 Jul 19. PMID: 33693055; PMCID: PMC7943172.
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