Ring finger protein 103

Ring finger protein 103 (RNF103) is a protein that in humans is encoded by the RNF103 gene. This protein is part of the RING finger protein family, which is characterized by a unique structural motif that binds zinc.

Function

The RNF103 protein is a E3 ubiquitin-protein ligase that accepts ubiquitin from an E2 ubiquitin-conjugating enzyme in the form of a thioester and then directly transfers the ubiquitin to targeted substrates. This protein plays a role in the protein degradation pathway associated with the ubiquitin-proteasome system (UPS).

Clinical significance

Alterations in the RNF103 gene have been associated with various diseases. For example, mutations in this gene have been linked to neurodegenerative diseases due to the protein's role in the degradation of misfolded proteins.

Structure

The RNF103 protein contains a RING finger domain, which is a protein structural domain of zinc finger that contains a C3HC4 amino acid motif which binds two zinc cations.

See also

• RING finger domain
• Ubiquitin-proteasome system
• E3 ubiquitin-protein ligase

References

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