Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice

Christopher S. Potter, Kathleen A. Silva, Victoria E. Kennedy, Timothy M. Stearns, Harm HogenEsch, John P. Sundberg

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Mice with mutations in SHANK-associated RH domain interactor (Sharpin) develop a hypereosinophilic auto-inflammatory disease known as chronic proliferative dermatitis. Affected mice have increased apoptosis in the keratinocytes of the skin, oesophagus and forestomach driven by extrinsic TNF receptor-mediated apoptotic signalling pathways. FAS receptor signalling is an extrinsic apoptotic signalling mechanism frequently involved in inflammatory skin diseases. Compound mutations in Sharpin and Fas or Fasl were created to determine whether these death domain proteins influenced the cutaneous phenotype in Sharpin null mice. Both Sharpin/Fas and Sharpin/Fasl compound mutant mice developed an auto-inflammatory phenotype similar to that seen in Sharpin null mice, indicating that initiation of apoptosis by FAS signalling is likely not involved in the pathogenesis of this disease.

Original languageEnglish (US)
JournalExperimental Dermatology
DOIs
StateAccepted/In press - 2017
Externally publishedYes

Fingerprint

Apoptosis
Skin
Dermatitis
Phenotype
Mutation
Tumor Necrosis Factor Receptors
Keratinocytes
Skin Diseases
Esophagus
sharpin
Proteins

Keywords

  • cpdm
  • Fas
  • Fasl
  • SHARPIN
  • Apoptosis

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Dermatology

Cite this

Potter, C. S., Silva, K. A., Kennedy, V. E., Stearns, T. M., HogenEsch, H., & Sundberg, J. P. (Accepted/In press). Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice. Experimental Dermatology. https://doi.org/10.1111/exd.13289

Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice. / Potter, Christopher S.; Silva, Kathleen A.; Kennedy, Victoria E.; Stearns, Timothy M.; HogenEsch, Harm; Sundberg, John P.

In: Experimental Dermatology, 2017.

Research output: Contribution to journalArticle

Potter, Christopher S. ; Silva, Kathleen A. ; Kennedy, Victoria E. ; Stearns, Timothy M. ; HogenEsch, Harm ; Sundberg, John P. / Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice. In: Experimental Dermatology. 2017.
@article{ca42a829f2b74d5ba9677fe28ccabb0f,
title = "Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice",
abstract = "Mice with mutations in SHANK-associated RH domain interactor (Sharpin) develop a hypereosinophilic auto-inflammatory disease known as chronic proliferative dermatitis. Affected mice have increased apoptosis in the keratinocytes of the skin, oesophagus and forestomach driven by extrinsic TNF receptor-mediated apoptotic signalling pathways. FAS receptor signalling is an extrinsic apoptotic signalling mechanism frequently involved in inflammatory skin diseases. Compound mutations in Sharpin and Fas or Fasl were created to determine whether these death domain proteins influenced the cutaneous phenotype in Sharpin null mice. Both Sharpin/Fas and Sharpin/Fasl compound mutant mice developed an auto-inflammatory phenotype similar to that seen in Sharpin null mice, indicating that initiation of apoptosis by FAS signalling is likely not involved in the pathogenesis of this disease.",
keywords = "cpdm, Fas, Fasl, SHARPIN, Apoptosis",
author = "Potter, {Christopher S.} and Silva, {Kathleen A.} and Kennedy, {Victoria E.} and Stearns, {Timothy M.} and Harm HogenEsch and Sundberg, {John P.}",
year = "2017",
doi = "10.1111/exd.13289",
language = "English (US)",
journal = "Experimental Dermatology",
issn = "0906-6705",
publisher = "Wiley-Blackwell",

}

TY - JOUR

T1 - Loss of FAS/FASL signalling does not reduce apoptosis in Sharpin null mice

AU - Potter, Christopher S.

AU - Silva, Kathleen A.

AU - Kennedy, Victoria E.

AU - Stearns, Timothy M.

AU - HogenEsch, Harm

AU - Sundberg, John P.

PY - 2017

Y1 - 2017

N2 - Mice with mutations in SHANK-associated RH domain interactor (Sharpin) develop a hypereosinophilic auto-inflammatory disease known as chronic proliferative dermatitis. Affected mice have increased apoptosis in the keratinocytes of the skin, oesophagus and forestomach driven by extrinsic TNF receptor-mediated apoptotic signalling pathways. FAS receptor signalling is an extrinsic apoptotic signalling mechanism frequently involved in inflammatory skin diseases. Compound mutations in Sharpin and Fas or Fasl were created to determine whether these death domain proteins influenced the cutaneous phenotype in Sharpin null mice. Both Sharpin/Fas and Sharpin/Fasl compound mutant mice developed an auto-inflammatory phenotype similar to that seen in Sharpin null mice, indicating that initiation of apoptosis by FAS signalling is likely not involved in the pathogenesis of this disease.

AB - Mice with mutations in SHANK-associated RH domain interactor (Sharpin) develop a hypereosinophilic auto-inflammatory disease known as chronic proliferative dermatitis. Affected mice have increased apoptosis in the keratinocytes of the skin, oesophagus and forestomach driven by extrinsic TNF receptor-mediated apoptotic signalling pathways. FAS receptor signalling is an extrinsic apoptotic signalling mechanism frequently involved in inflammatory skin diseases. Compound mutations in Sharpin and Fas or Fasl were created to determine whether these death domain proteins influenced the cutaneous phenotype in Sharpin null mice. Both Sharpin/Fas and Sharpin/Fasl compound mutant mice developed an auto-inflammatory phenotype similar to that seen in Sharpin null mice, indicating that initiation of apoptosis by FAS signalling is likely not involved in the pathogenesis of this disease.

KW - cpdm

KW - Fas

KW - Fasl

KW - SHARPIN

KW - Apoptosis

UR - http://www.scopus.com/inward/record.url?scp=85018999907&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85018999907&partnerID=8YFLogxK

U2 - 10.1111/exd.13289

DO - 10.1111/exd.13289

M3 - Article

JO - Experimental Dermatology

JF - Experimental Dermatology

SN - 0906-6705

ER -