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IQ motif containing H (IQCH) is a testis-specific protein involved in sperm development and function. The protein contains an IQ motif, a calmodulin-binding domain enabling calcium-mediated signal transduction, as well as other enzyme-like domains. It interacts with calmodulin and regulates key molecules including RNA-binding proteins required for spermatogenesis (in particular, HNRPAB). Deficiency or mutation of IQCH leads to abnormal sperm morphology, impaired motility, defective acrosome and mitochondrial development, and consequently male infertility. IQCH has four isoforms in humans produced by alternative splicing. No evidence exists for pharmacological targeting of IQCH or its role as a direct therapeutic target. Its predominant biological activity is constrained to male germ cell differentiation and reproductive function.
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