Mouse anti-Human sapiens (Human) RNH1 Monoclonal Antibody

Function

Ribonuclease inhibitor which inhibits RNASE1, RNASE2 and angiogenin (ANG) (PubMed:12578357, PubMed:14515218, PubMed:3219362, PubMed:3243277, PubMed:3470787, PubMed:9050852). May play a role in redox homeostasis (PubMed:17292889). Required to inhibit the cytotoxic tRNA ribonuclease activity of ANG in the cytoplasm in absence of stress (PubMed:23843625, PubMed:32510170). Relocates to the nucleus in response to stress, relieving inhibition of ANG in the cytoplasm, and inhibiting the angiogenic activity of ANG in the nucleus (PubMed:23843625)

Subcellular Location

Cytoplasm; Nucleus

Disease Association

Encephalitis, acute, infection-induced, 12 (IIAE12) : An autosomal recessive disorder apparent in infancy or early childhood, and characterized by acute encephalopathy triggered by viral infections and febrile illness. Neurologic features of the acute episodes include seizures, hemiplegia, decreased consciousness, hypotonia, abnormal posturing, feeding problems, and respiratory insufficiency. Disease severity is variable, ranging from death to normal neurologic outcomes. [Disease susceptibility is associated with variants affecting the gene represented in this entry]

Subunit

Forms high-affinity heterodimers with RNASE1, ANG and RNASE2

• Lee F.S. et al. Primary structure of human placental ribonuclease inhibitor. — Biochemistry (1988)
• Schneider R. et al. The primary structure of human ribonuclease/angiogenin inhibitor (RAI) discloses a novel highly diversified protein superfamily with a common repetitive module. — EMBO J. (1988)
• Huang G.H. et al. Expression of a human ribonuclease inhibitor variant in Escherichia coli and silkworm insect cell (Bombyx mori). — Sheng Wu Hua Xue Yu Sheng Wu Wu Li Xue Bao (2003)
• Bechtel S. et al. The full-ORF clone resource of the German cDNA consortium. — BMC Genomics (2007)
• The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). — Genome Res. (2004)
• Nadano D. et al. Purification and characterization of human brain ribonuclease inhibitor. — Arch. Biochem. Biophys. (1994)
• Crevel-Thieffry I. et al. Characterisation of a tryptic peptide from human placental ribonuclease inhibitor which inhibits ribonuclease activity. — Biochim. Biophys. Acta (1992)
• Shapiro R. et al. Human placental ribonuclease inhibitor abolishes both angiogenic and ribonucleolytic activities of angiogenin. — Proc. Natl. Acad. Sci. U.S.A. (1987)