Article

PPa1 insufficiency drives lysosomal storage disease and inflammatory macrophage expansion in the bone marrow

2026-03-18

Abstract excerpt

<h4>ABSTRACT</h4> Inorganic pyrophosphatase-1 (PPa1) is an essential enzyme proposed to limit accumulation of the ubiquitous metabolic byproduct inorganic pyrophosphate, yet its role beyond a general housekeeping function remains poorly understood. We generated viable hypomorphic alleles of PPa1 through random mutagenesis in mice and unexpectedly found that PPa1 insufficiency causes a lysosomal storage disease...

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Literature Corpus work: 6d12a15b-31b5-5fd8-a9b8-b834c413af02
DOI: 10.64898/2026.03.16.712247

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