SUMO4

Function

Ubiquitin-like protein which can be covalently attached to target lysines as a monomer. Does not seem to be involved in protein degradation and may modulate protein subcellular localization, stability or activity. Upon oxidative stress, conjugates to various anti-oxidant enzymes, chaperones, and stress defense proteins. May also conjugate to NFKBIA, TFAP2A and FOS, negatively regulating their transcriptional activity, and to NR3C1, positively regulating its transcriptional activity. Covalent attachment to its substrates requires prior activation by the E1 complex SAE1-SAE2 and linkage to the E2 enzyme UBE2I.

Involvement in disease

Type 1 diabetes mellitus 5

T1D5

A form of diabetes mellitus, a multifactorial disorder of glucose homeostasis that is characterized by susceptibility to ketoacidosis in the absence of insulin therapy. Clinical features are polydipsia, polyphagia and polyuria which result from hyperglycemia-induced osmotic diuresis and secondary thirst. These derangements result in long-term complications that affect the eyes, kidneys, nerves, and blood vessels.

None

Disease susceptibility may be associated with variants affecting the gene represented in this entry. Valine at position 55 results in greater NFKB1 transcriptional activity and IL12B expression and may be associated with susceptibility to insulin-dependent diabetes mellitus.

Post-translational modifications

In contrast to SUMO1, SUMO2 and SUMO3, seems to be insensitive to sentrin-specific proteases due to the presence of Pro-90. This may impair processing to mature form and conjugation to substrates.

Sequence Similarities

Belongs to the ubiquitin family. SUMO subfamily.

Tissue Specificity

Expressed mainly in adult and embryonic kidney. Expressed at various levels in immune tissues, with the highest expression in the lymph node and spleen.

Alternative names

SMT3H4, SUMO4, Small ubiquitin-related modifier 4, SUMO-4, Small ubiquitin-like protein 4

Database links

swissprot:Q6EEV6 entrezGene:6612 entrezGene:387082 entrezGene:6613 swissprot:P61956 swissprot:P55854 omim:608829 omim:602231 omim:603042