VRK1

Function

Serine/threonine kinase involved in Golgi disassembly during the cell cycle: following phosphorylation by PLK3 during mitosis, required to induce Golgi fragmentation. Acts by mediating phosphorylation of downstream target protein. Phosphorylates 'Thr-18' of p53/TP53 and may thereby prevent the interaction between p53/TP53 and MDM2. Phosphorylates casein and histone H3. Phosphorylates BANF1: disrupts its ability to bind DNA, reduces its binding to LEM domain-containing proteins and causes its relocalization from the nucleus to the cytoplasm. Phosphorylates ATF2 which activates its transcriptional activity.

Involvement in disease

Pontocerebellar hypoplasia 1A

PCH1A

A disorder characterized by an abnormally small cerebellum and brainstem, central and peripheral motor dysfunction from birth, gliosis and spinal cord anterior horn cells degeneration resembling infantile spinal muscular atrophy. Additional features include muscle hypotonia, congenital contractures and respiratory insufficiency that is evident at birth.

None

The disease is caused by variants affecting the gene represented in this entry.

Post-translational modifications

Autophosphorylated at various serine and threonine residues. Autophosphorylation does not impair its ability to phosphorylate p53/TP53. Phosphorylation by PLK3 leads to induction of Golgi fragmentation during mitosis.

Sequence similarities

Belongs to the protein kinase superfamily. CK1 Ser/Thr protein kinase family. VRK subfamily.

Tissue specificity

Widely expressed. Highly expressed in fetal liver, testis and thymus.

Cellular localization

• Cytoplasm
• Nucleus
• Cytoplasm
• Cytoskeleton
• Spindle
• Dispersed throughout the cell but not located on mitotic spindle or chromatids during mitosis.

Alternative names

• Serine/threonine-protein kinase VRK1
• Vaccinia-related kinase 1
• VRK1

Database links

• entrezGene:7443
• omim:602168
• swissprot:Q99986

Target type

Proteins

Molecular weight

45476Da