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A reference for what the literature reports. Not medical advice.

© 2026 CatalystPrivacy TermsReference data under the licences listed on the attribution page.

Proteins/UNIPROT:Q9BQ65

USB1

Protein·UNIPROT:Q9BQ65

Open in the graph

3'-5' RNA exonuclease that trims the 3' end of oligo(U) and oligo(A) tracts of the pre-U6 small nuclear RNA (snRNA) molecule, leading to the formation of a mature U6 snRNA 3' end-terminated with a 2',3'-cyclic phosphate. Participates in the U6 snRNA 3' end processing that prevents U6 snRNA degradation. In addition also removes uridines from the 3' end of U6atac snRNA and possibly the vault RNA VTRNA1-1..

Function from UniProtKB (Q9BQ65), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

2 rows

dyskeratosis congenita has a germline basis in USB1DiseaseGenCC HGNC:25792|MONDO:0015780Inferred only
poikiloderma with neutropenia has a germline basis in USB1DiseaseGenCC HGNC:25792|MONDO:0011405Inferred only

Statements

1 statement

1 statement · 1 reference database · Subject: USB1 · Sources: Hetionet

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SMAD4 interacts with USB1ProteinHetionet hetionet:interacts:4089-79650Inferred only

Reactions

2 reactions

a 3'-end uridylyl-uridine-RNAa 3'-end 2',3'-cyclophospho-uridine-RNA+uridine
Catalysed by USB1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:46052CC BY 4.0Reference data
a 3'-end uridylyl-adenosine-RNAa 3'-end 2',3'-cyclophospho-uridine-RNA+adenosine
Catalysed by USB1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:67896CC BY 4.0Reference data

Other names

7 names

Resolves to: USB1

Also called: 3'-5' RNA exonuclease USB1C16orf57hMpn1hUsb1Mpn1Mutated in PN protein 1Mutated in poikiloderma with neutropenia protein 1

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