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Corticospinal Neuron Dysfunction and Degeneration in ALS: Testing the Role of Corticomotor Connectivity in Motor Neuron Disease

Award Number: R01NS119508
ORGANIZATION: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE
OPDIV: NIH
AWARD CLASS: DISCRETIONARY
AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)
PERIOD OF PERFORMANCE START DATE: 12/01/2020
PERIOD OF PERFORMANCE END DATE: 11/30/2025

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Corticospinal Neuron Dysfunction and Degeneration in ALS: Testing the Role of Corticomotor Connectivity in Motor Neuron Disease - Abstract (Summary): In patients with amyotrophic lateral sclerosis (ALS) and the related motor neuron disease (MND) primary lateral sclerosis (PLS), deficits in motor control occur as a consequence of the degeneration of corticospinal neurons (CSNs). ALS is more common than PLS, and genetically more complex, with familial forms associated with causal mutations in over 30 ALS-related genes. In these ALS mice, however, dysfunction and degeneration of CSNs have not been carefully examined, and data implicating corticospinal (CS) circuits in these model systems of ALS is surprisingly limited. One reason for this may be the very different pattern of connectivity between CSNs and spinal MNs in humans vs. mice. In humans, CS axons located in the ventral and lateral funiculi form direct connections with both MNs (cortico-motoneuronal (CM) connections) and interneurons. In contrast, CS axons in mice are located mainly in the dorsal funiculus and only form indirect connections with MNs through pre-motor interneurons. Therefore, we will use PlexinA1 mutant mice which have CM connections together with ALS mouse models to analyze CS circuits. Our central hypothesis is that progressive defects in CS circuitry in ALS mice will be exacerbated by the establishment of CM connections. In Aim 1, we will determine formation of CS circuits in ALS mouse models with CM connections. In Aim 2, we will determine function of CS circuits in ALS mouse models with CM connections. In Aim 3, we will examine skilled movements in ALS mouse models with CM connections. These studies will provide a model system to study mechanisms of CS degeneration in ALS/PLS, and to test novel therapeutics targeting upper motor neuron dysfunction in these disorders.


Issue Date FY	Funding FY	Legal Entity Name	Legal Entity Address	Legal Entity City	Legal Entity State	Legal Entity Zip Code	Legal Entity COUNTY	Legal Entity COUNTRY	Assistance Listing	Award Code	Budget Year	Action Date	Action Type	Action Amount

Issue Date FY: 2026 ( Subtotal = $0 )
2026	2025	THE WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	5	9/16/2026	NON-COMPETING CONTINUATION	$0
														Subtotal = $0

Issue Date FY: 2025 ( Subtotal = $652,030 )
2025	2025	THE WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	5	11/18/2024	NON-COMPETING CONTINUATION	$586,828
2025	2025	THE WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	001	5	7/23/2025	NON-COMPETING CONTINUATION	$65,202
														Subtotal = $652,030

Issue Date FY: 2024 ( Subtotal = $645,302 )
2024	2024	THE WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	001	4	4/11/2024	NON-COMPETING CONTINUATION	$46,568
2024	2024	THE WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	4	11/22/2023	NON-COMPETING CONTINUATION	$598,734
														Subtotal = $645,302

Issue Date FY: 2023 ( Subtotal = $681,914 )
2023	2023	WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC, THE	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	001	3	1/30/2023	NON-COMPETING CONTINUATION	$68,190
2023	2023	WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC, THE	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	3	11/15/2022	NON-COMPETING CONTINUATION	$613,724
														Subtotal = $681,914

Issue Date FY: 2022 ( Subtotal = $693,379 )
2022	2022	WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC, THE	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	2	11/6/2021	NON-COMPETING CONTINUATION	$624,041
2022	2022	WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC, THE	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	001	2	3/21/2022	NON-COMPETING CONTINUATION	$69,338
														Subtotal = $693,379

Issue Date FY: 2021 ( Subtotal = $726,701 )
2021	2021	WINIFRED MASTERSON BURKE MEDICAL RESEARCH INSTITUTE, INC, THE	785 MAMARONECK AVE	WHITE PLAINS	NY	10605	WESTCHESTER	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	1	11/24/2020	NEW	$726,701
														Subtotal = $726,701

Grand Total All Awards = $3,399,326

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Corticospinal Neuron Dysfunction and Degeneration in ALS: Testing the Role of Corticomotor Connectivity in Motor Neuron Disease

Award Number: R01NS119508

ORGANIZATION: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

OPDIV: NIH

AWARD CLASS: DISCRETIONARY

AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)

PERIOD OF PERFORMANCE START DATE: 12/01/2020

PERIOD OF PERFORMANCE END DATE: 11/30/2025

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