Proteins/UNIPROT:Q9NVV4

MTPAP

ProteinUNIPROT:Q9NVV4

Polymerase that creates the 3' poly(A) tail of mitochondrial transcripts. Can use all four nucleotides, but has higher activity with ATP and UTP (in vitro). Plays a role in replication-dependent histone mRNA degradation. May be involved in the terminal uridylation of mature histone mRNAs before their degradation is initiated. Might be responsible for the creation of some UAA stop codons which are not encoded in mtDNA..

Function from UniProtKB (Q9NVV4), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

mitochondrial disease has a germline basis in MTPAPDiseaseGenCC HGNC:25532|MONDO:0044970Inferred only
spastic ataxia 4 has a germline basis in MTPAPDiseaseGenCC HGNC:25532|MONDO:0013354Inferred only

Statements

14 statements

14 statements · 3 reference databases · Subject: MTPAP · Sources: Hetionet, Reactome, UniProt

Order

MS4A7 covaries with MTPAPProteinHetionet hetionet:covaries:55149-58475Inferred only
MTPAP interacts with AZI2ProteinHetionet hetionet:interacts:55149-64343Inferred only
CALCOCO2 interacts with MTPAPProteinHetionet hetionet:interacts:10241-55149Inferred only
TBK1 interacts with MTPAPProteinHetionet hetionet:interacts:29110-55149Inferred only
UBC interacts with MTPAPProteinHetionet hetionet:interacts:55149-7316Inferred only
MTPAP participates in Metabolism of RNAPathwayReactome R-HSA-8953854Inferred only
MTPAP participates in Mitochondrial mRNA modificationPathwayReactome R-HSA-9937008Inferred only
HACD3 decreases the expression of MTPAPProteinHetionet hetionet:regulates:51495-55149Inferred only
RPP38 decreases the expression of MTPAPProteinHetionet hetionet:regulates:10557-55149Inferred only
TGDS decreases the expression of MTPAPProteinHetionet hetionet:regulates:23483-55149Inferred only
XRCC4 increases the expression of MTPAPProteinHetionet hetionet:regulates:7518-55149Inferred only
YME1L1 decreases the expression of MTPAPProteinHetionet hetionet:regulates:10730-55149Inferred only

Show the remaining 2

MTPAP requires the cofactor magnesium(2+)CompoundUniProt Q9NVV4Inferred only
MTPAP requires the cofactor Mn(2+)CompoundUniProt Q9NVV4Inferred only

Reactions

3 reactions

0.18ATP+0.3CHEBI:17677+0.34GTP+0.18UTPdiphosphate(3-)+MAM02847n
Catalysed by POLRMT & POLR3A & POLR1C & POLR3G & POLR2D & POLR1A & POLR3D & POLR1D & POLR2C & POLR2E & POLR2B & POLR2I & PAPOLA &

Other names

7 names

Resolves to: MTPAP

Also called: APAPPAP-associated domain-containing protein 1PAPD1Polynucleotide adenylyltransferaseTerminal uridylyltransferase 1TUTase 1

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