PNPT1 Human

Polyribonucleotide Nucleotidyltransferase 1 Human Recombinant
Cat. No.
BT12082
Source
Escherichia Coli.
Synonyms
Polyribonucleotide Nucleotidyltransferase 1, Polynucleotide Phosphorylase-Like Protein, Polynucleotide Phosphorylase 1, 3-5 RNA Exonuclease OLD35, PNPase Old-35, EC 2.7.7.8, PNPase 1, COXPD13, DFNB70, PNPASE, OLD35, Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial, Deafness, Autosomal Recessive 70, Polynucleotide Phosphorylase, 3-5 RNA Exonuclease, EC 2.7.7, Old-35, Polyribonucleotide nucleotidyltransferase 1, mitochondrial, 3'-5' RNA exonuclease OLD35, PNPase old-35.
Appearance
Sterile filtered colorless solution.
Purity
Greater than 85.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
Shipped with Ice Packs
In Stock

Description

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

Product Specs

Introduction
Polyribonucleotide nucleotidyltransferase 1, also known as PNPT1, is a protein primarily located in the mitochondrial intermembrane space. It plays a crucial role in transporting RNA into mitochondria. Genetic mutations in PNPT1 have been linked to combined oxidative phosphorylation deficiency-13 and autosomal recessive nonsyndromic deafness-70. Pseudogenes related to PNPT1 have been identified on chromosomes 3 and 7.
Description
Recombinant human PNPT1, produced in E. coli, is a single, non-glycosylated polypeptide chain consisting of 761 amino acids (46-783 a.a.). It has a molecular mass of 83.3 kDa. The protein is fused to a 23 amino acid His-tag at the N-terminus and purified using proprietary chromatographic methods.
Physical Appearance
Clear, colorless solution, sterile filtered.
Formulation
PNPT1 protein solution at a concentration of 0.25 mg/ml in phosphate buffered saline (pH 7.4) with 10% glycerol and 1 mM DTT.
Stability
For short-term storage (2-4 weeks), the product can be stored at 4°C. For extended storage, freeze the product at -20°C. Adding a carrier protein (0.1% HSA or BSA) is recommended for long-term storage. Avoid repeated freeze-thaw cycles.
Purity
Purity exceeds 85.0% as determined by SDS-PAGE.
Synonyms
Polyribonucleotide Nucleotidyltransferase 1, Polynucleotide Phosphorylase-Like Protein, Polynucleotide Phosphorylase 1, 3-5 RNA Exonuclease OLD35, PNPase Old-35, EC 2.7.7.8, PNPase 1, COXPD13, DFNB70, PNPASE, OLD35, Polyribonucleotide Nucleotidyltransferase 1, Mitochondrial, Deafness, Autosomal Recessive 70, Polynucleotide Phosphorylase, 3-5 RNA Exonuclease, EC 2.7.7, Old-35, Polyribonucleotide nucleotidyltransferase 1, mitochondrial, 3'-5' RNA exonuclease OLD35, PNPase old-35.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSAVAVDLG NRKLEISSGK LARFADGSAV VQSGDTAVMV TAVSKTKPSP SQFMPLVVDY RQKAAAAGRI PTNYLRREIG TSDKEILTSR IIDRSIRPLF PAGYFYDTQV LCNLLAVDGV NEPDVLAING ASVALSLSDI PWNGPVGAVR IGIIDGEYVV NPTRKEMSSS TLNLVVAGAP KSQIVMLEAS AENILQQDFC HAIKVGVKYT QQIIQGIQQL VKETGVTKRT PQKLFTPSPE IVKYTHKLAM ERLYAVFTDY EHDKVSRDEA VNKIRLDTEE QLKEKFPEAD PYEIIESFNV VAKEVFRSIV LNEYKRCDGR DLTSLRNVSC EVDMFKTLHG SALFQRGQTQ VLCTVTFDSL ESGIKSDQVI TAINGIKDKN FMLHYEFPPY ATNEIGKVTG LNRRELGHGA LAEKALYPVI PRDFPFTIRV TSEVLESNGS SSMASACGGS LALMDSGVPI SSAVAGVAIG LVTKTDPEKG EIEDYRLLTD ILGIEDYNGD MDFKIAGTNK GITALQADIK LPGIPIKIVM EAIQQASVAK KEILQIMNKT ISKPRASRKE NGPVVETVQV PLSKRAKFVG PGGYNLKKLQ AETGVTISQV DEETFSVFAP TPSAMHEARD FITEICKDDQ EQQLEFGAVY TATITEIRDT GVMVKLYPNM TAVLLHNTQL DQRKIKHPTA LGLEVGQEIQ VKYFGRDPAD GRMRLSRKVL QSPATTVVRT LNDRSSIVMG EPISQSSSNS Q.

Product Science Overview

Structure and Localization

PNPT1 is a phosphate-dependent 3’-to-5’ exoribonuclease that catalyzes the phosphorolysis of single-stranded polyribonucleotides in a processive manner . The enzyme is predominantly localized in the mitochondrial intermembrane space, where it is involved in the import of RNA into mitochondria . The human recombinant form of PNPT1 is produced in E. coli and consists of a single, non-glycosylated polypeptide chain containing 761 amino acids, with a molecular mass of approximately 83.3 kDa .

Function

PNPT1 is a component of the mitochondrial degradosome (mtEXO) complex, which degrades 3’ overhang double-stranded RNA in an ATP-dependent manner . This enzyme is involved in the degradation of non-coding mitochondrial transcripts (MT-ncRNA) and tRNA-like molecules . Additionally, PNPT1 has been implicated in numerous RNA metabolic processes, including RNA processing and degradation .

Clinical Significance

Mutations in the PNPT1 gene have been associated with several disorders, including combined oxidative phosphorylation deficiency-13 and autosomal recessive nonsyndromic deafness-70 . These conditions highlight the importance of PNPT1 in maintaining mitochondrial function and RNA metabolism.

Research and Applications

The human recombinant form of PNPT1 is used in various research applications to study its role in RNA metabolism and mitochondrial function. The enzyme’s activity can be analyzed in vitro to understand its biochemical properties and its involvement in RNA processing pathways .

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