RNH1
entrez
6050
canonical
uniprot
P13489
canonical
ensembl
ENSG00000023191
resolver
ensembl
ENSG00000276230
resolver
ensp
ENSP00000346402
resolver
ensp
ENSP00000348515
resolver
ensp
ENSP00000380729
resolver
ensp
ENSP00000380738
resolver
ensp
ENSP00000380739
resolver
ensp
ENSP00000416589
resolver
ensp
ENSP00000433999
resolver
ensp
ENSP00000435594
resolver
ensp
ENSP00000478664
resolver
ensp
ENSP00000479004
resolver
ensp
ENSP00000479966
resolver
ensp
ENSP00000480036
resolver
ensp
ENSP00000484572
resolver
ensp
ENSP00000487753
resolver
ensp
ENSP00000487940
resolver
ensp
ENSP00000488734
resolver
ensp
ENSP00000573693
resolver
ensp
ENSP00000573695
resolver
ensp
ENSP00000573696
resolver
ensp
ENSP00000573700
resolver
ensp
ENSP00000573702
resolver
ensp
ENSP00000573703
resolver
ensp
ENSP00000573704
resolver
ensp
ENSP00000573707
resolver
ensp
ENSP00000573709
resolver
ensp
ENSP00000573710
resolver
ensp
ENSP00000573711
resolver
ensp
ENSP00000573712
resolver
ensp
ENSP00000573713
resolver
ensp
ENSP00000573714
resolver
ensp
ENSP00000573715
resolver
ensp
ENSP00000573716
resolver
ensp
ENSP00000573717
resolver
ensp
ENSP00000573718
resolver
ensp
ENSP00000573719
resolver
ensp
ENSP00000573720
resolver
ensp
ENSP00000573722
resolver
ensp
ENSP00000573725
resolver
ensp
ENSP00000573726
resolver
ensp
ENSP00000573727
resolver
ensp
ENSP00000573728
resolver
ensp
ENSP00000573729
resolver
ensp
ENSP00000573730
resolver
ensp
ENSP00000573731
resolver
ensp
ENSP00000573732
resolver
ensp
ENSP00000573734
resolver
ensp
ENSP00000573735
resolver
ensp
ENSP00000573736
resolver
ensp
ENSP00000573737
resolver
ensp
ENSP00000573738
resolver
ensp
ENSP00000573739
resolver
ensp
ENSP00000573740
resolver
ensp
ENSP00000610573
resolver
ensp
ENSP00000610574
resolver
ensp
ENSP00000610575
resolver
ensp
ENSP00000610578
resolver
ensp
ENSP00000610580
resolver
ensp
ENSP00000642524
resolver
ensp
ENSP00000642529
resolver
ensp
ENSP00000642530
resolver
ensp
ENSP00000642533
resolver
ensp
ENSP00000642534
resolver
ensp
ENSP00000642548
resolver
ensp
ENSP00000642549
resolver
ensp
ENSP00000642558
resolver
ensp
ENSP00000642559
resolver
ensp
ENSP00000642560
resolver
ensp
ENSP00000642561
resolver
ensp
ENSP00000642566
resolver
ensp
ENSP00000642567
resolver
ensp
ENSP00000642569
resolver
ensp
ENSP00000642570
resolver
ensp
ENSP00000642571
resolver
enst
ENST00000354420
resolver
enst
ENST00000356187
resolver
enst
ENST00000397604
resolver
enst
ENST00000397614
resolver
enst
ENST00000397615
resolver
enst
ENST00000438658
resolver
enst
ENST00000533410
resolver
enst
ENST00000534797
resolver
enst
ENST00000612988
resolver
enst
ENST00000617351
resolver
enst
ENST00000618184
resolver
enst
ENST00000619599
resolver
enst
ENST00000621211
resolver
enst
ENST00000632527
resolver
enst
ENST00000632954
resolver
enst
ENST00000633287
resolver
enst
ENST00000903634
resolver
enst
ENST00000903636
resolver
enst
ENST00000903637
resolver
enst
ENST00000903641
resolver
enst
ENST00000903643
resolver
enst
ENST00000903644
resolver
enst
ENST00000903645
resolver
enst
ENST00000903648
resolver
enst
ENST00000903650
resolver
enst
ENST00000903651
resolver
enst
ENST00000903652
resolver
enst
ENST00000903653
resolver
enst
ENST00000903654
resolver
enst
ENST00000903655
resolver
enst
ENST00000903656
resolver
enst
ENST00000903657
resolver
enst
ENST00000903658
resolver
enst
ENST00000903659
resolver
enst
ENST00000903660
resolver
enst
ENST00000903661
resolver
enst
ENST00000903663
resolver
enst
ENST00000903666
resolver
enst
ENST00000903667
resolver
enst
ENST00000903668
resolver
enst
ENST00000903669
resolver
enst
ENST00000903670
resolver
enst
ENST00000903671
resolver
enst
ENST00000903672
resolver
enst
ENST00000903673
resolver
enst
ENST00000903675
resolver
enst
ENST00000903676
resolver
enst
ENST00000903677
resolver
enst
ENST00000903678
resolver
enst
ENST00000903679
resolver
enst
ENST00000903680
resolver
enst
ENST00000903681
resolver
enst
ENST00000940514
resolver
enst
ENST00000940515
resolver
enst
ENST00000940516
resolver
enst
ENST00000940519
resolver
enst
ENST00000940521
resolver
enst
ENST00000972465
resolver
enst
ENST00000972470
resolver
enst
ENST00000972471
resolver
enst
ENST00000972474
resolver
enst
ENST00000972475
resolver
enst
ENST00000972489
resolver
enst
ENST00000972490
resolver
enst
ENST00000972499
resolver
enst
ENST00000972500
resolver
enst
ENST00000972501
resolver
enst
ENST00000972502
resolver
enst
ENST00000972507
resolver
enst
ENST00000972508
resolver
enst
ENST00000972510
resolver
enst
ENST00000972511
resolver
enst
ENST00000972512
resolver
genesymbol
RNH1
resolver
genesymbol_syn
PRI
resolver
genesymbol_syn
RNH
resolver
hgnc
HGNC:10074
resolver
uniprot
A0A140VJT8
resolver
uniprot
Q8IZK8
resolver
uniprot
Q96FD7
resolver
uniprot
Q9BQ80
resolver
uniprot
Q9UDK6
resolver
uniprot_entry
A0A140VJT8_HUMAN
resolver
uniprot_entry
RINI_HUMAN
resolver
uniprot_sec
Q8IZK8
resolver
uniprot_sec
Q96FD7
resolver
uniprot_sec
Q9BQ80
resolver
uniprot_sec
Q9UDK6
resolver
amino_acid_sequence
MSLDIQSLDIQCEELSDARWAELLPLLQQCQVVRLDDCGLTEARCKDISSALRVNPALAELNLRSNELGDVGVHCVLQGLQTPSCKIQKLSLQNCCLTGAGCGVLSSTLRTLPTLQELHLSDNLLGDAGLQLLCEGLLDPQCRLEKLQLEYCSLSAASCEPLASVLRAKPDFKELTVSNNDINEAGVRVLCQGLKDSPCQLEALKLESCGVTSDNCRDLCGIVASKASLRELALGSNKLGDVGMAELCPGLLHPSSRLRTLWIWECGITAKGCGDLCRVLRAKESLKELSLAGNELGDEGARLLCETLLEPGCQLESLWVKSCSFTAACCSHFSSVLAQNRFLLELQISNNRLEDAGVRELCQGLGQPGSVLRVLWLADCDVSDSSCSSLAATLLANHSLRELDLSNNCLGDAGILQLVESVRQPGCLLEQLVLYDIYWSEEMEDRLQALEKDKPSLRVIS
uniprot
proteins
disease_involvement
DISEASE: Encephalitis, acute, infection-induced, 12 (IIAE12) [MIM:620461]: 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. {ECO:0000269|PubMed:36935417, ECO:0000269|PubMed:37191094}. Note=Disease susceptibility is associated with variants affecting the gene represented in this entry.
uniprot
proteins
function
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). {ECO:0000269|PubMed:12578357, ECO:0000269|PubMed:14515218, ECO:0000269|PubMed:17292889, ECO:0000269|PubMed:23843625, ECO:0000269|PubMed:3219362, ECO:0000269|PubMed:3243277, ECO:0000269|PubMed:32510170, ECO:0000269|PubMed:3470787, ECO:0000269|PubMed:9050852}.
uniprot
proteins
mass_dalton
49973
uniprot
proteins
mutagenesis
MUTAGEN 262; /note="W->A: Binding affinity decreased 5000-fold over the wild type for RNASE2; when associated with A-264 and A-319."; /evidence="ECO:0000269|PubMed:12578357"; MUTAGEN 264; /note="W->A: Substantially decreased binding affinity for RNASE2. Binding affinity decreased 5000-fold over the wild type for RNASE2; when associated with A-262 and A-319."; /evidence="ECO:0000269|PubMed:12578357"; MUTAGEN 319; /note="W->A: Substantially decreased binding affinity for RNASE2. Binding affinity decreased 5000-fold over the wild type for RNASE2; when associated with A-262 and A-264."; /evidence="ECO:0000269|PubMed:12578357"; MUTAGEN 376; /note="W->A: 40-fold reduction in binding affinity for RNASE2."; /evidence="ECO:0000269|PubMed:15755456"; MUTAGEN 435..436; /note="YD->AA: Substantially decreases binding affinity for RNASE1, ANG and RNASE2."; /evidence="ECO:0000269|PubMed:10413501, ECO:0000269|PubMed:12578357"; MUTAGEN 458; /note="R->A: 25-fold reduction in binding affinity for RNASE2."; /evidence="ECO:0000269|PubMed:15755456"; MUTAGEN 461; /note="Missing: A significant decrease in binding affinity with RNASE1, slight decrease for the ANG ligand, no real change in binding affinity for RNASE2."; /evidence="ECO:0000269|PubMed:12578357, ECO:0000269|PubMed:9050852
uniprot
proteins
post_translational_modification
PTM: The N-terminus is blocked. {ECO:0000269|PubMed:8037455}.; PTM: At least 30 of the 32 cysteine residues are in the reduced form. {ECO:0000269|PubMed:3219362}.
uniprot
proteins
pubmed
10413501
uniprot
proteins
pubmed
12578357
uniprot
proteins
pubmed
14515218
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15755456
uniprot
proteins
pubmed
1633192
uniprot
proteins
pubmed
17292889
uniprot
proteins
pubmed
17350650
uniprot
proteins
pubmed
17974005
uniprot
proteins
pubmed
18669648
uniprot
proteins
pubmed
19690332
uniprot
proteins
pubmed
20068231
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
22223895
uniprot
proteins
pubmed
22814378
uniprot
proteins
pubmed
23843625
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
3219362
uniprot
proteins
pubmed
3243277
uniprot
proteins
pubmed
32510170
uniprot
proteins
pubmed
3470787
uniprot
proteins
pubmed
36935417
uniprot
proteins
pubmed
37191094
uniprot
proteins
pubmed
8037455
uniprot
proteins
pubmed
9050852
uniprot
proteins
pubmed
9311977
uniprot
proteins
sequence_length
461
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Cytoplasm {ECO:0000269|PubMed:23843625, ECO:0000305|PubMed:3219362}. Nucleus {ECO:0000269|PubMed:23843625}. Note=Localizes in the cytoplasm in absence of stress; translocates to the nucleus in response to stress. {ECO:0000269|PubMed:23843625}.
uniprot
proteins