Has an E3 ubiquitin ligase activity. Ubiquitinates DTNBP1 (dysbindin) and promotes its degradation. May play a significant role in mediating the biological activity of the HIV-1 Tat protein in vivo. Binds specifically to the activation domain of HIV-1 Tat and can also interact with the HIV-2 and EIAV Tat proteins in vivo.
Spleen, thymus, prostate, testis, ovary, intestine, colon and skeletal muscle.
Protein modification; protein ubiquitination.
Involvement in disease
Limb-girdle muscular dystrophy 2H (LGMD2H) [MIM:254110]: An autosomal recessive degenerative myopathy characterized by pelvic girdle, shoulder girdle and quadriceps muscle weakness. Clinical phenotype and severity are highly variable. Disease progression is slow and most patients remain ambulatory into the sixth decade of life. Note=The disease is caused by mutations affecting the gene represented in this entry. Bardet-Biedl syndrome 11 (BBS11) [MIM:209900]: A syndrome characterized by usually severe pigmentary retinopathy, early-onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. Bardet-Biedl syndrome inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for clinical manifestation of some forms of the disease. Note=The disease is caused by mutations affecting the gene represented in this entry.
Belongs to the TRIM/RBCC family. Contains 1 B box-type zinc finger. Contains 5 NHL repeats. Contains 1 RING-type zinc finger.
Cytoplasm. Localized in cytoplasmic bodies, often located around the nucleus.