SHPRH

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Description

The SHPRH (SNF2 histone linker PHD RING helicase) is a protein-coding gene located on chromosome 6.

SHPRH (also known as E3 ubiquitin-protein ligase SHPRH) is an enzyme encoded by the SHPRH gene in humans. It is ubiquitously expressed and contains motifs characteristic of DNA repair proteins, transcription factors, and helicases. SHPRH acts as a nucleosome-stimulated ATPase and a nucleosome-E3 ubiquitin ligase.

SHPRH acts as an E3 ubiquitin ligase in DNA repair. In response to DNA damage, it receives ubiquitin from the UBE2N-UBE2V2 E2 complex and attaches it to Lys-164 of PCNA, which has been previously monoubiquitinated by UBE2A/B-RAD18. This process facilitates the formation of non-canonical polyubiquitin chains linked through Lys-63.

SHPRH is also known as RAD5, bA545I5.2.

Associated Diseases

• dermatophytosis
• hemoglobin D disease
• hereditary persistence of fetal hemoglobin-sickle cell disease syndrome
• dominant beta-thalassemia
• hemoglobin C-beta-thalassemia syndrome
• delta-beta-thalassemia
• cancer
• hemoglobin E disease
• hemoglobin H disease
• hemoglobin E-beta-thalassemia syndrome
• alpha-thalassemia-myelodysplastic syndrome
• beta-thalassemia-X-linked thrombocytopenia syndrome