https://transhumanist.ru/index.php?action=history&feed=atom&title=TPP2TPP2 - История изменений2026-06-23T11:08:37ZИстория изменений этой страницы в викиMediaWiki 1.43.6https://transhumanist.ru/index.php?title=TPP2&diff=3946&oldid=prevOdysseusBot: Новая страница: «Tripeptidyl-peptidase 2 (EC 3.4.14.10) (TPP-2) (Tripeptidyl aminopeptidase) (Tripeptidyl-peptidase II) (TPP-II) ==Publications== {{medline-entry |title=Early-on...»2021-04-29T18:53:04Z<p>Новая страница: «Tripeptidyl-peptidase 2 (EC 3.4.14.10) (TPP-2) (Tripeptidyl aminopeptidase) (Tripeptidyl-peptidase II) (TPP-II) ==Publications== {{medline-entry |title=Early-on...»</p>
<p><b>Новая страница</b></p><div>Tripeptidyl-peptidase 2 (EC 3.4.14.10) (TPP-2) (Tripeptidyl aminopeptidase) (Tripeptidyl-peptidase II) (TPP-II)<br />
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==Publications==<br />
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{{medline-entry<br />
|title=Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency.<br />
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/25414442<br />
|abstract=Autoimmune cytopenia is a frequent manifestation of primary immunodeficiencies. Two siblings presented with Evans syndrome, viral infections, and progressive leukopenia. DNA available from one patient showed a homozygous frameshift mutation in tripeptidyl peptidase II ([[TPP2]]) abolishing protein expression. [[TPP2]] is a serine exopeptidase involved in extralysosomal peptide degradation. Its deficiency in mice activates cell death programs and premature senescence. Similar to cells from naïve, uninfected [[TPP2]]-deficient mice, patient cells showed increased major histocompatibility complex I expression and most CD8( ) T-cells had a senescent CCR7-CD127(-)CD28(-)CD57( ) phenotype with poor proliferative responses and enhanced staurosporine-induced apoptosis. T-cells showed increased expression of the effector molecules perforin and interferon-γ with high expression of the transcription factor T-bet. Age-associated B-cells with a CD21(-) CD11c( ) phenotype expressing T-bet were increased in humans and mice, combined with antinuclear antibodies. Moreover, markers of senescence were also present in human and murine [[TPP2]]-deficient fibroblasts. Telomere lengths were normal in patient fibroblasts and granulocytes, and low normal in lymphocytes, which were compatible with activation of stress-induced rather than replicative senescence programs. [[TPP2]] deficiency is the first primary immunodeficiency linking premature immunosenescence to severe autoimmunity. Determination of senescent lymphocytes should be part of the diagnostic evaluation of children with refractory multilineage cytopenias. <br />
|mesh-terms=* Aging<br />
* Aminopeptidases<br />
* Anemia, Hemolytic, Autoimmune<br />
* Animals<br />
* Apoptosis<br />
* Base Sequence<br />
* CD8-Positive T-Lymphocytes<br />
* Child<br />
* Child, Preschool<br />
* Consanguinity<br />
* Dipeptidyl-Peptidases and Tripeptidyl-Peptidases<br />
* Female<br />
* Fibroblasts<br />
* Frameshift Mutation<br />
* Gene Expression<br />
* Humans<br />
* Immunologic Deficiency Syndromes<br />
* Male<br />
* Mice<br />
* Mice, Knockout<br />
* Molecular Sequence Data<br />
* Perforin<br />
* Serine Endopeptidases<br />
* Siblings<br />
* T-Box Domain Proteins<br />
* T-Lymphocytes<br />
* Thrombocytopenia<br />
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|full-text-url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4463807<br />
}}</div>OdysseusBot