InterPro domain: IPR037299

This entry represents the MATH (meprin and TRAF homology) domain found in TRIM37, which is a member of the tripartite motif (RING, B-Box, and coiled-coil domains) family and has E3 ubiquitin ligase activity toward itself and histone H2A protein [ 1 ]. TRIM37 localizes to peroxisomes [ 2 ]. Mutations in the human TRIM37 gene (also known as MUL) cause Mulibrey (muscle-liver-brain-eye) nanism, a rare growth disorder of prenatal onset characterized by dysmorphic features, pericardial constriction and hepatomegaly [ 3 ].

TRIM37 contains a tripartite motif and a MATH domain C-terminal to the coiled-coil domain.

1. TRIM37 defective in mulibrey nanism is a novel RING finger ubiquitin E3 ligase. Exp. Cell Res. 308, 146-55
2. The TRIM37 gene encodes a peroxisomal RING-B-box-coiled-coil protein: classification of mulibrey nanism as a new peroxisomal disorder. Am. J. Hum. Genet. 70, 1215-28
3. Novel mutations in the TRIM37 gene in Mulibrey Nanism. Hum. Mutat. 23, 522