TRIM37
This gene encodes a member of the tripartite motif (TRIM) family, whose members are involved in diverse cellular functions such as developmental patterning and oncogenesis. The TRIM motif includes zinc-binding domains, a RING finger region, a B-box motif and a coiled-coil domain. The RING finger and B-box domains chelate zinc and might be involved in protein-protein and/or protein-nucleic acid interactions. The gene mutations are associated with mulibrey (muscle-liver-brain-eye) nanism, an autosomal recessive disorder that involves several tissues of mesodermal origin. [provided by RefSeq, Mar 2016]
Full Name
TRIM37 Gene(Protein Coding) Tripartite Motif Containing 37
Function
E3 ubiquitin-protein ligase required to prevent centriole reduplication (PubMed:15885686, PubMed:23769972).
Probably acts by ubiquitinating positive regulators of centriole reduplication (PubMed:23769972).
Mediates monoubiquitination of 'Lys-119' of histone H2A (H2AK119Ub), a specific tag for epigenetic transcriptional repression: associates with some Polycomb group (PcG) multiprotein PRC2-like complex and mediates repression of target genes (PubMed:25470042).
Has anti-HIV activity (PubMed:24317724).
Biological Process
Biological Process aggresome assembly Source:UniProtKB1 Publication
Biological Process histone H2A monoubiquitination Source:UniProtKB1 Publication
Biological Process histone H2A-K119 monoubiquitination Source:UniProtKB1 Publication
Biological Process negative regulation of centriole replication Source:UniProtKB1 Publication
Biological Process negative regulation of NF-kappaB transcription factor activity Source:UniProtKB1 Publication
Biological Process negative regulation of transcription by RNA polymerase II Source:UniProtKB1 Publication
Biological Process positive regulation of DNA-binding transcription factor activity Source:UniProtKB1 Publication
Biological Process positive regulation of NF-kappaB transcription factor activity Source:UniProtKB1 Publication
Biological Process protein autoubiquitination Source:UniProtKB1 Publication
Cellular Location
Cytoplasm, perinuclear region
Peroxisome
Found in vesicles of the peroxisome. Aggregates as aggresomes, a perinuclear region where certain misfolded or aggregated proteins are sequestered for proteasomal degradation.
Involvement in disease
Mulibrey nanism (MUL):
An autosomal recessive growth disorder characterized by severe growth failure of prenatal onset, constrictive pericardium and progressive cardiomyopathy, facial dysmorphism, and failure of sexual maturation. Additional clinical features include hepatomegaly, muscle hypotonia, J-shaped sella turcica, yellowish dots in the ocular fundi, hypoplasia of various endocrine glands, insulin resistance with type 2 diabetes, and an increased risk for Wilms' tumor.