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G-patch domain and KOW motifs-containing protein (GPKOW) is an RNA-binding protein involved in pre-mRNA splicing, specifically acting as a component of the minor spliceosome that facilitates splicing of U12-type introns in pre-mRNAs. It interacts with protein kinase A and protein kinase X and is associated with the spliceosome. Mutations in the GPKOW gene have been linked to disorders such as microhydranencephaly and adrenoleukodystrophy. GPKOW contains both a G-patch domain and two KOW motifs, structural elements that are often present in proteins mediating RNA interactions and catalytic steps in RNA processing[2][5][6].
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