Serpins in Caenorhabditis elegans

Cliff J. Luke; Mark T. Miedel; Linda P. O’Reilly; Allyson Wyatt; Ryan R. Knoerdel; Stephen C. Pak; Gary A. Silverman

doi:10.1007/978-3-319-22711-5_15

Serpins in Caenorhabditis elegans

Cliff J. Luke, Mark T. Miedel, Linda P. O’Reilly, Allyson Wyatt, Ryan R. Knoerdel, Stephen C. Pak, Gary A. Silverman

Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review

1 Scopus citations

Abstract

C. elegans is an extremely powerful model organism to study gene function and biology. The nematodes’ genetic tractability and high degree of genetic similarity with humans make it ideal to study the biologic role of serpins. The endogenous C. elegans serpins are most similar to the human clade B or intracellular serpin family and have a predominantly cytoplasmic distribution. Since the nematode genes often have functional orthologues in humans, the biological role of mammalian serpins can be extrapolated from C. elegans. Additionally, mutant-mammalian serpin genes resulting in serpinopathies can be introduced into C. elegans to determine the biological pathways that play a role in disease progression, as well as develop potential therapeutics to ameliorate the disease.

Original language	English
Title of host publication	The Serpin Family
Subtitle of host publication	Proteins with Multiple Functions in Health and Disease
Publisher	Springer International Publishing
Pages	253-268
Number of pages	16
ISBN (Electronic)	9783319227115
ISBN (Print)	9783319227108
DOIs	https://doi.org/10.1007/978-3-319-22711-5_15
State	Published - Jan 1 2015

Keywords

ATD
Caenorhabditis elegans
Cathepsin
Clade B (or intracellular) serpins
Intracellular serpin
Model organism
Necrotic cell death (or NCD) serpinopathies
SRP-1
SRP-2
SRP-3
SRP-6
SRP-7
α1-antitrypsin

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abstract = "C. elegans is an extremely powerful model organism to study gene function and biology. The nematodes{\textquoteright} genetic tractability and high degree of genetic similarity with humans make it ideal to study the biologic role of serpins. The endogenous C. elegans serpins are most similar to the human clade B or intracellular serpin family and have a predominantly cytoplasmic distribution. Since the nematode genes often have functional orthologues in humans, the biological role of mammalian serpins can be extrapolated from C. elegans. Additionally, mutant-mammalian serpin genes resulting in serpinopathies can be introduced into C. elegans to determine the biological pathways that play a role in disease progression, as well as develop potential therapeutics to ameliorate the disease.",

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AU - Luke, Cliff J.

AU - Miedel, Mark T.

AU - O’Reilly, Linda P.

AU - Wyatt, Allyson

AU - Knoerdel, Ryan R.

AU - Pak, Stephen C.

AU - Silverman, Gary A.

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N2 - C. elegans is an extremely powerful model organism to study gene function and biology. The nematodes’ genetic tractability and high degree of genetic similarity with humans make it ideal to study the biologic role of serpins. The endogenous C. elegans serpins are most similar to the human clade B or intracellular serpin family and have a predominantly cytoplasmic distribution. Since the nematode genes often have functional orthologues in humans, the biological role of mammalian serpins can be extrapolated from C. elegans. Additionally, mutant-mammalian serpin genes resulting in serpinopathies can be introduced into C. elegans to determine the biological pathways that play a role in disease progression, as well as develop potential therapeutics to ameliorate the disease.

AB - C. elegans is an extremely powerful model organism to study gene function and biology. The nematodes’ genetic tractability and high degree of genetic similarity with humans make it ideal to study the biologic role of serpins. The endogenous C. elegans serpins are most similar to the human clade B or intracellular serpin family and have a predominantly cytoplasmic distribution. Since the nematode genes often have functional orthologues in humans, the biological role of mammalian serpins can be extrapolated from C. elegans. Additionally, mutant-mammalian serpin genes resulting in serpinopathies can be introduced into C. elegans to determine the biological pathways that play a role in disease progression, as well as develop potential therapeutics to ameliorate the disease.

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KW - Cathepsin

KW - Clade B (or intracellular) serpins

KW - Intracellular serpin

KW - Model organism

KW - Necrotic cell death (or NCD) serpinopathies

KW - SRP-1

KW - SRP-2

KW - SRP-3

KW - SRP-6

KW - SRP-7

KW - α1-antitrypsin

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SN - 9783319227108

SP - 253

EP - 268

BT - The Serpin Family

PB - Springer International Publishing

ER -

Serpins in Caenorhabditis elegans

Abstract

Keywords

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