Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase

Polynucleotide phosphorylase (PNPase) is a phosphorolytic RNA exonuclease highly conserved throughout evolution. Human PNPase (hPNPase) is located in mitochondria and is essential for mitochondrial function and homeostasis. Not surprisingly, mutations in the <i>PNPT1</i> gene, encoding h...

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Main Authors: Federica A. Falchi, Roberto Pizzoccheri, Federica Briani
Format: Article
Language:English
Published: MDPI AG 2022-01-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/23/3/1652
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author Federica A. Falchi
Roberto Pizzoccheri
Federica Briani
author_facet Federica A. Falchi
Roberto Pizzoccheri
Federica Briani
author_sort Federica A. Falchi
collection DOAJ
description Polynucleotide phosphorylase (PNPase) is a phosphorolytic RNA exonuclease highly conserved throughout evolution. Human PNPase (hPNPase) is located in mitochondria and is essential for mitochondrial function and homeostasis. Not surprisingly, mutations in the <i>PNPT1</i> gene, encoding hPNPase, cause serious diseases. hPNPase has been implicated in a plethora of processes taking place in different cell compartments and involving other proteins, some of which physically interact with hPNPase. This paper reviews hPNPase RNA binding and catalytic activity in relation with the protein structure and in comparison, with the activity of bacterial PNPases. The functions ascribed to hPNPase in different cell compartments are discussed, highlighting the gaps that still need to be filled to understand the physiological role of this ancient protein in human cells.
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spelling doaj.art-62fc5e0057034be1858ba3d9254b63582023-11-23T16:44:06ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-01-01233165210.3390/ijms23031652Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide PhosphorylaseFederica A. Falchi0Roberto Pizzoccheri1Federica Briani2Dipartimento di Bioscienze, Università degli Studi di Milano, 20133 Milano, ItalyDipartimento di Bioscienze, Università degli Studi di Milano, 20133 Milano, ItalyDipartimento di Bioscienze, Università degli Studi di Milano, 20133 Milano, ItalyPolynucleotide phosphorylase (PNPase) is a phosphorolytic RNA exonuclease highly conserved throughout evolution. Human PNPase (hPNPase) is located in mitochondria and is essential for mitochondrial function and homeostasis. Not surprisingly, mutations in the <i>PNPT1</i> gene, encoding hPNPase, cause serious diseases. hPNPase has been implicated in a plethora of processes taking place in different cell compartments and involving other proteins, some of which physically interact with hPNPase. This paper reviews hPNPase RNA binding and catalytic activity in relation with the protein structure and in comparison, with the activity of bacterial PNPases. The functions ascribed to hPNPase in different cell compartments are discussed, highlighting the gaps that still need to be filled to understand the physiological role of this ancient protein in human cells.https://www.mdpi.com/1422-0067/23/3/1652polyribonucleotide phosphorylasePNPase<i>PNPT1</i>RNA decayRNA stabilityRNA binding protein
spellingShingle Federica A. Falchi
Roberto Pizzoccheri
Federica Briani
Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
International Journal of Molecular Sciences
polyribonucleotide phosphorylase
PNPase
<i>PNPT1</i>
RNA decay
RNA stability
RNA binding protein
title Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
title_full Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
title_fullStr Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
title_full_unstemmed Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
title_short Activity and Function in Human Cells of the Evolutionary Conserved Exonuclease Polynucleotide Phosphorylase
title_sort activity and function in human cells of the evolutionary conserved exonuclease polynucleotide phosphorylase
topic polyribonucleotide phosphorylase
PNPase
<i>PNPT1</i>
RNA decay
RNA stability
RNA binding protein
url https://www.mdpi.com/1422-0067/23/3/1652
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AT federicabriani activityandfunctioninhumancellsoftheevolutionaryconservedexonucleasepolynucleotidephosphorylase