About: Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors

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About: Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : wasabi.inria.fr associated with source document(s)

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
title	Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors
Creator	Auburger, Georg Gispert, Suzana Hamann, Andrea Jendrach, Marina Klinkenberg, Michael Adler, Thure Busch, Dirk Walter, Michael Wittig, Ilka Brandt, Ulrich Grzmil, Pawel Bü Chel, Finja Drö Se, Stefan Hrabé De Angelis, Martin Koob, Sebastian Mittelbronn, Michel Osiewacz, Heinz Parganlija, Dajana Reichert, Andreas Zell, Andreas
source	PMC
abstract	The caseinolytic peptidase P (CLPP) is conserved from bacteria to humans. In the mitochondrial matrix, it multimerizes and forms a macromolecular proteasome-like cylinder together with the chaperone CLPX. In spite of a known relevance for the mitochondrial unfolded protein response, its substrates and tissue-specific roles are unclear in mammals. Recessive CLPP mutations were recently observed in the human Perrault variant of ovarian failure and sensorineural hearing loss. Here, a first characterization of CLPP null mice demonstrated complete female and male infertility and auditory deficits. Disrupted spermatogenesis already at the spermatid stage and ovarian follicular differentiation failure were evident. Reduced pre-/post-natal survival and marked ubiquitous growth retardation contrasted with only light impairment of movement and respiratory activities. Interestingly, the mice showed resistance to ulcerative dermatitis. Systematic expression studies detected up-regulation of other mitochondrial chaperones, accumulation of CLPX and mtDNA as well as inflammatory factors throughout tissues. T-lymphocytes in the spleen were activated. Thus, murine Clpp deletion represents a faithful Perrault model. The disease mechanism probably involves deficient clearance of mitochondrial components and inflammatory tissue destruction.
has issue date	2013-12-15(xsd:dateTime)
bibo:doi	10.1093/hmg/ddt338
bibo:pmid	23851121
has license	no-cc
sha1sum (hex)	75e701c7f4cd0a33dc6e4dd6d56093f194f972bf
schema:url	https://doi.org/10.1093/hmg/ddt338
resource representing a document's title	Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors
has PubMed Central identifier	PMC7108587
has PubMed identifier	23851121
schema:publication	Hum Mol Genet
resource representing a document's body	covid:75e701c7f4cd0a33dc6e4dd6d56093f194f972bf#body_text
is schema:about of	named entity 'mutations' named entity 'T-lymphocytes' covid:arg/75e701c7f4cd0a33dc6e4dd6d56093f194f972bf named entity 'humans' named entity 'substrates' named entity 'Systematic' named entity 'deletion' named entity 'activated' named entity 'CLPP' named entity 'Present' named entity 'mitochondrial' named entity 'sensorineural hearing loss' named entity 'mice' named entity 'mitochondrial unfolded protein response' named entity 'spermatogenesis' named entity 'mitochondrial matrix' named entity 'sensorineural hearing loss' named entity 'up-regulation' named entity 'inflammatory' named entity 'ulcerative dermatitis' named entity 'spermatid' named entity 'peptidase' named entity 'proteasome' named entity 'T-lymphocytes' named entity 'macromolecular' named entity 'null mice' named entity 'peptidase' named entity 'growth retardation' named entity 'CLPP' named entity 'antibody' named entity 'mtDNA' named entity 'T-cells' named entity 'neurodegeneration' named entity 'heterozygous' named entity 'CLPP' named entity 'CLPP' named entity 'prokaryotes' named entity 'chaperone' named entity 'E. coli' named entity 'Clpp' named entity 'infertility' named entity 'mice' named entity 'male infertility' named entity 'ribosomes' named entity 'downregulated' named entity 'Thermo Scientific' named entity 'F-statistic' named entity 'Clpp' named entity 'OPA1' named entity 'OPA1' named entity 'CD25' named entity 'tissue samples' named entity 'Rnf213' named entity 'Cox1' named entity 'frataxin' named entity 'osmium tetroxide' named entity 'control factor' named entity 'follicles' named entity 'gonad' named entity 'secondary antibody' named entity 'mitochondrial matrix' named entity 'Clpp'

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