KHDC4/BBP-like, KH-domain type I

Definition

This entry represents an eukaryotic type I KH domain found in a group of proteins involved in pre-mRNA splicing, such as KH domain-containing BBP from fungi, KH homology domain-containing protein 4 (KHDC4, also known as BLOM7) and Splicing factor 1 from animals, animal quaking proteins such as human QKI, and homologues from plants, including RIK. BBP/SF1 family members include the branchpoint binding proteins (BBP) from yeast and their mammalian homologue, splicing factor 1 (SF1). They are required for pre-spliceosome formation, which is the first step of pre-mRNA splicing [[cite:PMID:12374752], [cite:PMID:10775271]]. BBP binds the conserved RNA sequence UACUAAC, called the branchpoint motif, found near the 3' end of yeast introns PMID:21189690. Besides its function in pre-mRNA splicing, BBP is also involved in nuclear retention of pre-mRNA PMID:10775271. Mammalian SF1, also known as ZFM1, may act as transcription repressor PMID:9660765. KHDC4 interacts with the PRP19C/Prp19 complex/NTC/Nineteen complex which is part of the spliceosome. It binds preferentially RNA with A/C rich sequences and poly-C stretches. KHDC4 contains two type I K homology (KH) RNA-binding domains [[cite:PMID:23144703], [cite:PMID:19641227]]. RIK1 interacts with rough sheath2 (RS2), which is a myb domain protein required to repress knox genes during leaf development PMID:16243907. Human QKI is involved in various cellular processes, such as mRNA storage into stress granules, apoptosis, lipid deposition, interferon response, glial cell fate and development [[cite:PMID:25768908], [cite:PMID:31829086], [cite:PMID:34428287], [cite:PMID:37379838]]. The core KH domain is accompanied by additional α-helix and β-sheet motifs at the C terminus.

6 human proteins with this domain

Each is a reviewed human protein in the Seroatlas serome and a potential autoantibody target; this domain groups them into one antibody-relevant category. Every entry links to its own record.

Download CSV (6 proteins: gene, accession, name)

• KHDC4
• KHDRBS1
• KHDRBS2
• KHDRBS3
• QKI
• SF1