ID:PAPD1_HUMAN DESCRIPTION: RecName: Full=Poly(A) RNA polymerase, mitochondrial; Short=PAP; EC=2.7.7.19; AltName: Full=PAP-associated domain-containing protein 1; AltName: Full=Polynucleotide adenylyltransferase; AltName: Full=Terminal uridylyltransferase 1; Short=TUTase 1; AltName: Full=mtPAP; Flags: Precursor; FUNCTION: 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. CATALYTIC ACTIVITY: ATP + RNA(n) = diphosphate + RNA(n+1). COFACTOR: Magnesium or manganese. BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=0.1 mM for ATP; KM=0.7 mM for UTP; SUBUNIT: Homodimer. INTERACTION: Self; NbExp=3; IntAct=EBI-2556166, EBI-2556166; Q9QYP6:Azi2 (xeno); NbExp=2; IntAct=EBI-2556166, EBI-6115874; Q13137:CALCOCO2; NbExp=2; IntAct=EBI-2556166, EBI-739580; Q9UHD2:TBK1; NbExp=2; IntAct=EBI-2556166, EBI-356402; SUBCELLULAR LOCATION: Cytoplasm. Mitochondrion. TISSUE SPECIFICITY: Ubiquitous, with stronger expression in tissues with high energy requirements: heart, brain, and skeletal muscle. DISEASE: Defects in MTPAP are the cause of spastic ataxia autosomal recessive type 4 (SPAX4) [MIM:613672]. A slowly progressive neurodegenerative disease characterized by cerebellar ataxia, spastic paraparesis, dysarthria, and optic atrophy. Note=Affected individuals exhibit a drastic decrease in poly(A) tail length of representative mitochondrial mRNA transcripts, including COX1 and RNA14 (PubMed:20970105). SIMILARITY: Belongs to the DNA polymerase type-B-like family. SIMILARITY: Contains 1 PAP-associated domain.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q9NVV4
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
