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Human PNPT1 Recombinant Protein (RPPB2147)

SKU:
RPPB2147
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
Q8TCS8
Research Area:
Enzymes
€309
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Description

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Product Name:Human PNPT1 Recombinant Protein
Product Code:RPPB2147
Size:10µg
Species:Human
Target:PNPT1
Synonyms:Polyribonucleotide Nucleotidyltransferase 1, PolynucleotidePhosphorylase-Like Protein, Polynucleotide Phosphorylase 1, 3-5 RNA ExonucleaseOLD35, PNPase Old-35, EC 2.7.7.8, PNPase 1, COXPD13, DFNB70, PNPASE, OLD35, PolyribonucleotideNucleotidyltransferase 1, Mitochondrial, Deafness, Autosomal Recessive 70, PolynucleotidePhosphorylase, 3-5 RNA Exonuclease, EC 2.7.7, Old-35, Polyribonucleotidenucleotidyltransferase 1, mitochondrial, 3'-5' RNA exonuclease OLD35, PNPaseold-35.
Source:Escherichia Coli
Physical Appearance:Sterile filtered colorless solution.
Formulation:PNPT1 protein solution (0.25mg/ml) containing Phosphatebuffered saline (pH7.4), 10% glycerol and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
Purity:Greater than 85.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSAVAVDLG NRKLEISSGK LARFADGSAV VQSGDTAVMV TAVSKTKPSPSQFMPLVVDY RQKAAAAGRI PTNYLRREIG TSDKEILTSR IIDRSIRPLF PAGYFYDTQV LCNLLAVDGVNEPDVLAING ASVALSLSDI PWNGPVGAVR IGIIDGEYVV NPTRKEMSSS TLNLVVAGAP KSQIVMLEASAENILQQDFC HAIKVGVKYT QQIIQGIQQL VKETGVTKRT PQKLFTPSPE IVKYTHKLAM ERLYAVFTDYEHDKVSRDEA VNKIRLDTEE QLKEKFPEAD PYEIIESFNV VAKEVFRSIV LNEYKRCDGR DLTSLRNVSCEVDMFKTLHG SALFQRGQTQ VLCTVTFDSL ESGIKSDQVI TAINGIKDKN FMLHYEFPPY ATNEIGKVTGLNRRELGHGA LAEKALYPVI PRDFPFTIRV TSEVLESNGS SSMASACGGS LALMDSGVPI SSAVAGVAIGLVTKTDPEKG EIEDYRLLTD ILGIEDYNGD MDFKIAGTNK GITALQADIK LPGIPIKIVM EAIQQASVAKKEILQIMNKT ISKPRASRKE NGPVVETVQV PLSKRAKFVG PGGYNLKKLQ AETGVTISQV DEETFSVFAPTPSAMHEARD FITEICKDDQ EQQLEFGAVY TATITEIRDT GVMVKLYPNM TAVLLHNTQL DQRKIKHPTALGLEVGQEIQ VKYFGRDPAD GRMRLSRKVL QSPATTVVRT LNDRSSIVMG EPISQSSSNS Q

Polyribonucleotide nucleotidyltransferase 1, also known as PNPT1 ispredominantly localized in the mitochondrial intermembrane space and is implicated inthe import of RNA to mitochondria. Mutations in PNPT1 have been connected withcombined oxidative phosphorylation deficiency-13 as well as autosomal recessivenonsyndromic deafness-70. Related pseudogenes have been found on chromosomes 3 & 7.

PNPT1 Human Recombinant produced in E.Coli is a single, non-glycosylatedpolypeptide chain containing 761 amino acids (46-783 a.a) and having amolecular mass of 83.3kDa.PNPT1 is fused to a 23 amino acid His-tag at N-terminus& purified by proprietary chromatographic techniques.

UniProt Protein Function:PNPT1: RNA-binding protein implicated in numerous RNA metabolic processes. Hydrolyzes single-stranded polyribonucleotides processively in the 3'-to-5' direction. Mitochondrial intermembrane factor with RNA-processing exoribonulease activity. Component of the mitochondrial degradosome (mtEXO) complex, that degrades 3' overhang double-stranded RNA with a 3'-to-5' directionality in an ATP-dependent manner. Required for correct processing and polyadenylation of mitochondrial mRNAs. Plays a role as a cytoplasmic RNA import factor that mediates the translocation of small RNA components, like the 5S RNA, the RNA subunit of ribonuclease P and the mitochondrial RNA-processing (MRP) RNA, into the mitochondrial matrix. Plays a role in mitochondrial morphogenesis and respiration; regulates the expression of the electron transport chain (ETC) components at the mRNA and protein levels. In the cytoplasm, shows a 3'-to-5' exoribonuclease mediating mRNA degradation activity; degrades c- myc mRNA upon treatment with IFNB1/IFN-beta, resulting in a growth arrest in melanoma cells. Regulates the stability of specific mature miRNAs in melanoma cells; specifically and selectively degrades miR-221, preferentially. Plays also a role in RNA cell surveillance by cleaning up oxidized RNAs. Binds to the RNA subunit of ribonuclease P, MRP RNA and miR-221 microRNA. Belongs to the polyribonucleotide nucleotidyltransferase family.
UniProt Protein Details:

Protein type:Mitochondrial; Transferase; RNA-binding; EC 2.7.7.8; Nucleotide Metabolism - pyrimidine; Nucleotide Metabolism - purine

Chromosomal Location of Human Ortholog: 2p15

Cellular Component: cytoplasm; mitochondrial intermembrane space; mitochondrion

Molecular Function:3'-5'-exoribonuclease activity; miRNA binding; poly(rG) binding; poly(U) binding; polyribonucleotide nucleotidyltransferase activity; protein binding

Biological Process: mRNA catabolic process; negative regulation of growth; protein homooligomerization; regulation of cellular respiration; RNA catabolic process; RNA polyadenylation

Disease: Combined Oxidative Phosphorylation Deficiency 13; Deafness, Autosomal Recessive 70

NCBI Summary:The protein encoded by this gene belongs to the evolutionary conserved polynucleotide phosphorylase family comprised of phosphate dependent 3'-to-5' exoribonucleases implicated in RNA processing and degradation. This enzyme is predominantly localized in the mitochondrial intermembrane space and is involved in import of RNA to mitochondria. Mutations in this gene have been associated with combined oxidative phosphorylation deficiency-13 and autosomal recessive nonsyndromic deafness-70. Related pseudogenes are found on chromosomes 3 and 7. [provided by RefSeq, Dec 2012]
UniProt Code:Q8TCS8
NCBI GenInfo Identifier:115502437
NCBI Gene ID:87178
NCBI Accession:Q8TCS8.2
UniProt Secondary Accession:Q8TCS8,Q53SU0, Q68CN1, Q7Z7D1, Q8IWX1, Q96T05, Q9BRU3 Q9BVX0,
UniProt Related Accession:Q8TCS8
Molecular Weight:85,951 Da
NCBI Full Name:Polyribonucleotide nucleotidyltransferase 1, mitochondrial
NCBI Synonym Full Names:polyribonucleotide nucleotidyltransferase 1
NCBI Official Symbol:PNPT1��
NCBI Official Synonym Symbols:OLD35; DFNB70; PNPASE; old-35; COXPD13��
NCBI Protein Information:polyribonucleotide nucleotidyltransferase 1, mitochondrial
UniProt Protein Name:Polyribonucleotide nucleotidyltransferase 1, mitochondrial
UniProt Synonym Protein Names:3'-5' RNA exonuclease OLD35; PNPase old-35; Polynucleotide phosphorylase 1; PNPase 1; Polynucleotide phosphorylase-like protein
UniProt Gene Name:PNPT1��
UniProt Entry Name:PNPT1_HUMAN

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