Dataset Information

Proteomic analysis of innate immune responses in Polg D257A macrophages challenged with Pseudomonas aeruginosa

ABSTRACT: Mitochondrial diseases (MtD) represent a significant public health challenge due to their heterogenous clinical presentation, often severe and progressive symptoms, and lack of effective therapies. Environmental exposures, such bacterial and viral infection, can further compromise mitochondrial function and exacerbate the progression of MtD. Infections in MtD patients more frequently progress to sepsis, pneumonia, and other detrimental inflammatory endpoints. However, the underlying immune alterations that enhance immunopathology in MtD remain unclear, constituting a key gap in knowledge that complicates treatment and increases mortality in this vulnerable population. This data set utilizes an in vitro assay using Pseudomonas aeruginosa (PA) infection of bone marrow derived macrophages (BMDMs) to clarify the molecular and cellular basis for innate immune hyperactivity in a mouse model of polymerase gamma (Polg)-related MtD. This dataset reports the proteome signatures of WT and Polg D257A mutant BMDMs at rest and 2-8 hours post infection with PA.

INSTRUMENT(S): Orbitrap Astral

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Cell Culture, Bone Marrow, Macrophage

DISEASE(S): Mitochondrial Metabolism Disease,Alpers-huttenlocher Syndrome

SUBMITTER: Phillip West

LAB HEAD: Phillip West

PROVIDER: PXD062877 | Pride | 2025-04-14

REPOSITORIES: Pride

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Dataset's files

Source:

			Action	DRS
	20240924_001_West_BMDM_2046_rep1.msf	Msf
	20240924_001_West_BMDM_2046_rep1.raw	Raw
	20240924_001_West_BMDM_2046_rep2.msf	Msf
	20240924_001_West_BMDM_2046_rep2.raw	Raw
	20240924_002_West_BMDM_2034_rep1.msf	Msf

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Publications

Caspase-11 drives macrophage hyperinflammation in models of Polg-related mitochondrial disease.

VanPortfliet Jordyn J JJ Lei Yuanjiu Y Ramanathan Muthumeena M Martinez Camila Guerra CG Wong Jessica J Stodola Tim J TJ Hoffmann Brian R BR Pflug Kathryn K Sitcheran Raquel R Kneeland Stephen C SC Murray Stephen A SA McGuire Peter J PJ Cannon Carolyn L CL West A Phillip AP

bioRxiv : the preprint server for biology 20250117

Mitochondrial diseases (MtD) represent a significant public health challenge due to their heterogenous clinical presentation, often severe and progressive symptoms, and lack of effective therapies. Environmental exposures, such bacterial and viral infection, can further compromise mitochondrial function and exacerbate the progression of MtD. Infections in MtD patients more frequently progress to sepsis, pneumonia, and other detrimental inflammatory endpoints. However, the underlying immune alter ...[more]

PMID: 38798587

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