RNase P, ELAC2, and additional unknown nucleases cleave L strand transcripts to release the tRNAs and an mRNA contained in the long polycistronic transcripts. Mitochondrial RNase P, comprising 3 protein subunits and no RNA moiety (Holzmann et al. 2008), endonucleolytically cleaves polycistronic mitochondrial transcripts at the 5' ends of the tRNA sequences (Sanchez et al. 2011, Howard et al. 2012, Vilardo et al. 2012, Li et al. 2015, Reinhard et al. 2015, Vilardo and Rossmanith 2015). A subcomplex of RNase P also functions as a tRNA methyltransferase and the SDR5C1 subunit is an amino acid and fatty acid dehydrogenase. Mutations in the SDR5C1 subunit of RNase P cause HSD10 disease, which is characterized by progressive neurodegeneration and cardiomyopathy (Vilardo and Rossmanith 2015) ELAC2 cleaves polycistronic mitochondrial transcripts at the 3' ends of the tRNA sequences (Brzezniak et al. 2011, Sanchez et al. 2011). Different isoforms of ELAC2 are present in the nucleus and mitochondria (Rossmanith 2011). Mutations in ELAC2 cause cardiac hypertrophy (Haack et al. 2013). Unknown nucleases also cleave the L strand transcript at a site 3' to MT-ND6 (reviewed in Van Haute et al. 2015).
RNase P, ELAC2, and additional unknown nucleases cleave H strand transcripts to release the various tRNAs, rRNAs, and mRNAs contained in the long polycistronic transcripts. Mitochondrial RNase P, comprising 3 protein subunits and no RNA moiety (Holzmann et al. 2008), endonucleolytically cleaves polycistronic mitochondrial transcripts at the 5' ends of the tRNA sequences (Sanchez et al. 2011, Howard et al. 2012, Vilardo et al. 2012, Li et al. 2015, Reinhard et al. 2015, Vilardo and Rossmanith 2015). A subcomplex of RNase P also functions as a tRNA methyltransferase and the SDR5C1 subunit is an amino acid and fatty acid dehydrogenase. Mutations in the SDR5C1 subunit of RNase P cause HSD10 disease, which is characterized by progressive neurodegeneration and cardiomyopathy (Vilardo and Rossmanith 2015) ELAC2 cleaves polycistronic mitochondrial transcripts at the 3' ends of the tRNA sequences (Brzezniak et al. 2011, Sanchez et al. 2011). Different isoforms of ELAC2 are present in the nucleus and mitochondria (Rossmanith 2011). Mutations in ELAC2 cause cardiac hypertrophy (Haack et al. 2013) and disorders of oxidative phosphorylation (reviewed in Van Haute et al. 2015). Unknown nucleases also cleave the H strand transcript at sites 5' to MT-CO3, 5' to MT-CO1, and 5' to MT-CYB (reviewed in Van Haute et al. 2015).
RHOH plays an important role in development of T lymphocytes and binds to several kinases involved in T lymphocyte activation, including: ZAP70 (Gu et al. 2006, Chae et al. 2010, Wang et al. 2011) LCK (Chae et al. 2010, Wang et al. 2011) CSK (Wang et al. 2010).
Additional RHOH interactors include: PAK1 (Wang et al. 2010; Tajadura Ortega et al. 2018) PAK2 (Tajadura Ortega et al. 2018) PAK4 (Tajadura Ortega et al. 2018) PAK5 (Wu and Frost 2006; Tajadura Ortega et al. 2018) PAK6 (Tajadura Ortega et al. 2018)
The following RHOH interactors have only been reported in a high throughput screen by Bagci et al. 2020, and are therefore annotated as candidate RHOH effectors: CAV1 (Bagci et al. 2020). DBN1 (Bagci et al. 2020) DBT (Bagci et al. 2020) FAM91A1 (Bagci et al. 2020) JUP (Bagci et al. 2020) LAMTOR1 (Bagci et al. 2020) MTR (Bagci et al. 2020) NIPSNAP2 (Bagci et al. 2020) NSFL1C (Bagci et al. 2020) OSBPL11 (Bagci et al. 2020) RAB7A (Bagci et al. 2020) RALGAPA1 (Bagci et al. 2020) ROCK1 (Bagci et al. 2020) ROCK2 (Bagci et al. 2020) SLC1A5 (Bagci et al. 2020) SLC4A7 (Bagci et al. 2020) STOM (Bagci et al. 2020) TFRC (Bagci et al. 2020) TMEM59 (Bagci et al. 2020) TUBA1B (Bagci et al. 2020) UACA (Bagci et al. 2020) VAMP3 (Bagci et al. 2020) VANGL1 (Bagci et al. 2020) VCP (Bagci et al. 2020) WDR11 (Bagci et al. 2020)