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The 3-hydroxyisobutyryl-Coenzyme A hydrolase pseudogene (ENSG00000249541, LOC100130748) is a non-functional genetic sequence located in the human genome that resembles the protein-coding gene for 3-hydroxyisobutyryl-CoA hydrolase (HIBCH) but does not produce an active enzyme or functional protein. As a pseudogene, it does not participate in metabolic pathways, biological regulation, or disease processes, nor is it a therapeutic target. Its presence is an artifact of genomic evolution and it is typically ignored in pharmacological research. For comparison, the HIBCH gene (not the pseudogene) encodes the mitochondrial enzyme 3-hydroxyisobutyryl-CoA hydrolase, which is involved in valine metabolism and is associated with metabolic diseases when mutated.
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