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Leucine-rich repeat- and IQ domain-containing protein 1 (LRRIQ1) is a conserved, nuclear-encoded mitochondrial protein primarily characterized by its structural domains: four leucine-rich repeats (LRRs), facilitating protein–protein interactions, and an IQ calmodulin-binding motif, which can bind calmodulin or similar proteins independently of calcium. LRRIQ1 is highly conserved across metazoans, with highest expression in the testis, specifically in germ cells and Leydig cells. In mice, knockout of Lrriq1 causes reduced sperm motility and fertility due to increased apoptosis in testicular germ cells, associated with decreased levels of inhibin B, a regulator of spermatogenesis. Human LRRIQ1 is genetically linked to certain hereditary conditions such as sclerosteosis 1 and autosomal recessive deafness, but there is no current evidence of its therapeutic targeting by drugs, nor is it established as a clinical biomarker or drug target.
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