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Altered central carbon metabolism in mitochondrial myopathy

Award Number: R01AR088052
ORGANIZATION: NATIONAL INSTITUTE OF ARTHRITIS & MUSCULOSKELETAL & SKIN DISEASES
OPDIV: NIH
AWARD CLASS: DISCRETIONARY
AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)
PERIOD OF PERFORMANCE START DATE: 08/10/2026
PERIOD OF PERFORMANCE END DATE: 07/31/2031

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Altered central carbon metabolism in mitochondrial myopathy - Project Summary Mitochondria are centrally involved in the conversion of nutrients into energy, and mitochondrial dysfunction impairs this process, resulting in altered metabolism. The skeletal muscle system is prominently affected in humans with mitochondrial disease, but a detailed understanding of metabolic reprogramming in the disease state is missing. Our past work has evaluated patient-derived cells and mice with mitochondrial myopathy and revealed alterations in glucose handling and lactate metabolism which regulate disease progression downstream of impaired hypoxic signaling. Our long term objective is to understand metabolic alterations in humans and animals with mitochondrial myopathy in order to engineer targeted therapies that will benefit patients. In Aim 1, we will establish a set of mouse models of mitochondrial myopathy associated with electron transport chain inhibition and characterize Hif1alpha signaling, disease progression and altered lactate metabolism using isotope tracing technology coupled with mass spectrometry. In Aim 2, we will investigate mechanisms underlying altered metabolism and establish if genetic, pharmacologic or gene therapy approaches can ameliorate disease progression in pre-clinical models. In Aim 3, we will examine glucose and lactate metabolism in human mitochondrial myopathy patients using isotope tracing technology, in order to understand if similar metabolic alterations are present and correlate with disease. Together, these aims will assess how changes in metabolism correlate with disease progression of mitochondrial myopathy in mice and humans. The results have the potential to identify new therapies targeting glucose and lactate metabolism which may be beneficial for patients suffering from mitochondrial myopathies.


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 = $549,608 )
2026	2026	THE UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER	5323 HARRY HINES BLVD	DALLAS	TX	75390	DALLAS	USA	Arthritis, Musculoskeletal and Skin Diseases Research	000	1	8/7/2026	NEW	$549,608
														Subtotal = $549,608

Grand Total All Awards = $549,608

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Altered central carbon metabolism in mitochondrial myopathy

Award Number: R01AR088052

ORGANIZATION: NATIONAL INSTITUTE OF ARTHRITIS & MUSCULOSKELETAL & SKIN DISEASES

OPDIV: NIH

AWARD CLASS: DISCRETIONARY

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

PERIOD OF PERFORMANCE START DATE: 08/10/2026

PERIOD OF PERFORMANCE END DATE: 07/31/2031

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