NOP56 : NOP56 ribonucleoprotein


Description

The NOP56 (NOP56 ribonucleoprotein) is a protein-coding gene located on chromosome 20.

The NOP56 gene provides instructions for creating a protein known as nucleolar protein 56, found primarily within the nucleus of nerve cells (neurons), particularly in the cerebellum, a brain region involved in coordinating movement. Nucleolar protein 56 forms a subunit of the ribonucleoprotein complex, which comprises proteins and RNA molecules. This complex is vital for the production of ribosomes, the cellular structures responsible for processing genetic instructions to create proteins. Within the NOP56 gene, in a region called intron 1, there is a repeated sequence of six DNA building blocks (nucleotides) known as a hexanucleotide (GGCCTG). This hexanucleotide typically repeats 3 to 14 times within intron 1, although its precise function is currently unknown.

NOP56 plays a key role in the early to middle stages of building the 60S ribosomal subunit, which is essential for protein synthesis. It's a core component of box C/D small nucleolar ribonucleoprotein (snoRNP) particles, which are involved in the processing of specific types of RNA called snoRNAs. NOP56 is required for the production of U3, U8, and U14 snoRNAs, which are critical for ribosome assembly. NOP56 is also part of the small subunit (SSU) processome, the first precursor of the small eukaryotic ribosomal subunit. During the assembly of the SSU processome in the nucleolus, NOP56 works alongside many other factors to ensure proper RNA folding, modifications, and rearrangements, ultimately leading to the creation of a functional ribosome.

NOP56 is also known as NOL5A, SCA36.

Associated Diseases


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