|
Colitis
|
0.010 |
AlteredExpression
|
disease |
BEFREE |
A dozen were shown to possess improved properties, among which inhibition of YO-PRO-1/TO-PRO-3 uptake and IL1β release upon BzATP activation of the receptor and dampening signs of DSS-induced colitis on mice, in comparison with reference antagonist GSK1370319A.
|
31525963 |
2020 |
|
Progeria
|
0.800 |
Biomarker
|
disease |
BEFREE |
<b>Abbreviations:</b> HGPS: Hutchinson-Gilford progeria syndrome; ZMPSTE24: Zinc metallopeptidase STE24; FTI: Farnesyltransferase inhibitors; VSMCs: Vascular smooth muscle cells; iPSC: Induced pluripotent stem cells; EC: Endothelial cells; hTERT: Human telomerase reverse transcriptase; VEGF: vascular endothelial growth factor; DAF-FM DA: 3-Amino, 4-aminomethyl-2',7'-difluorofluorescein diacetate; BMP4: Bone Morphogenetic Protein 4; mmqPCR: mono chrome multiplex PCR; SCG: single-copy gene; CSI: Cell shape index.
|
31411525 |
2019 |
|
Progeria
|
0.800 |
Biomarker
|
disease |
BEFREE |
Mice deficient in zinc metalloproteinase STE24 (<i>Zmpste24</i><sup>-/-</sup>) exhibit premature age-related musculoskeletal pathologies similar to those observed in children with Hutchinson-Gilford progeria syndrome (HGPS).
|
31312666 |
2019 |
|
Osteoporosis
|
0.120 |
Biomarker
|
disease |
BEFREE |
Moreover, upregulating the activity of Ca<sub>v</sub> 1.2 mitigated osteoporosis symptom in Zmpste24-/- mice.
|
31120193 |
2019 |
|
Cockayne Syndrome
|
0.030 |
Biomarker
|
disease |
BEFREE |
Similar structural and functional mitochondrial defects are common to other neurodegenerative diseases, such as Parkinson disease and Prion disease, and to a progeroid syndrome like HGPS.
|
30830726 |
2019 |
|
Progeria
|
0.800 |
Biomarker
|
disease |
BEFREE |
Defects in prelamin A processing by ZMPSTE24 result in premature aging disorders including Hutchinson Gilford Progeria Syndrome (HGPS) and related progeroid diseases.
|
30625386 |
2019 |
|
Premature aging syndrome
|
0.060 |
Biomarker
|
disease |
BEFREE |
Defects in prelamin A processing by ZMPSTE24 result in premature aging disorders including Hutchinson Gilford Progeria Syndrome (HGPS) and related progeroid diseases.
|
30625386 |
2019 |
|
Mandibuloacral dysostosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Here the case of a boy affected with mandibuloacral dysplasia and compound heterozygous mutations in ZMPSTE24 is presented.
|
30548811 |
2019 |
|
Progeria
|
0.800 |
Biomarker
|
disease |
BEFREE |
Moreover, in vivo analysis of the Zmpste24-/- HGPS mouse model demonstrates that treatment with a sclerostin-neutralizing antibody (SclAb), which targets an antagonist of canonical WNT/β-catenin signaling pathway, fully rescues the low bone mass phenotype to wild-type levels.
|
30001457 |
2018 |
|
Diabetes Mellitus, Non-Insulin-Dependent
|
0.010 |
GeneticVariation
|
disease |
BEFREE |
Clinically relevant ZMPSTE24 variants identified in the largest database of exomes sequences derived from T2D patients were characterized using the yeast model, revealing 14 partial loss-of-function variants, which were enriched among diabetes patients over 2-fold.
|
29526462 |
2018 |
|
Actinic keratosis
|
0.010 |
Biomarker
|
disease |
BEFREE |
These AKs were graded as PRO I in 18·6% of cases, PRO II in 30·5% and PRO III in 50·8%.
|
29526028 |
2019 |
|
Fatty Liver
|
0.110 |
Biomarker
|
disease |
BEFREE |
Aged and Zmpste24-deficient livers share several features, including nuclear lamina abnormalities, increased Foxa2 binding, de-repression of PPAR- and LXR-dependent gene expression, and fatty liver.
|
29484800 |
2018 |
|
Steatohepatitis
|
0.110 |
Biomarker
|
disease |
BEFREE |
Aged and Zmpste24-deficient livers share several features, including nuclear lamina abnormalities, increased Foxa2 binding, de-repression of PPAR- and LXR-dependent gene expression, and fatty liver.
|
29484800 |
2018 |
|
Mandibuloacral dysostosis
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The molecular defects associated with MAD are mutations in LMNA or ZMPSTE24 (FACE1) gene, causing type A or type B MAD, respectively.
|
29208544 |
2018 |
|
Progeria
|
0.800 |
Biomarker
|
disease |
BEFREE |
Premature senescence can be induced by different stress factors and occurs in mouse embryonic fibroblasts (MEFs) derived from Zmpste24 metalloproteinase‑deficient mice, a progeria mouse model of Hutchinson‑Gilford Progeria Syndrome.
|
28990109 |
2017 |
|
Influenza A
|
0.020 |
AlteredExpression
|
disease |
BEFREE |
The zinc metalloprotease ZMPSTE24 is a constitutively and ubiquitously expressed host restriction factor that is responsible for limiting infection by a broad spectrum of enveloped viruses, including influenza A, vesicular stomatitis, zika, ebola, Sindbis, cowpox, and vaccinia viruses, but not murine leukemia or adenovirus.
|
28594571 |
2017 |
|
Malignant Neoplasms
|
0.010 |
Biomarker
|
group |
BEFREE |
The goals of this one-year, culinary-based, pilot intervention were to: 1) decrease Pro-I biomarkers and increase anti-inflammatory (AI) cytokine, IL-10, by promoting AI food incorporation into BCS diets; and 2) examine intervention effects on cancer risk factors including body mass index (BMI) and circulating adipose stromal cells (ASCs).A total of 153 BCSs were recruited.
|
28342988 |
2017 |
|
Primary malignant neoplasm
|
0.010 |
Biomarker
|
group |
BEFREE |
The goals of this one-year, culinary-based, pilot intervention were to: 1) decrease Pro-I biomarkers and increase anti-inflammatory (AI) cytokine, IL-10, by promoting AI food incorporation into BCS diets; and 2) examine intervention effects on cancer risk factors including body mass index (BMI) and circulating adipose stromal cells (ASCs).A total of 153 BCSs were recruited.
|
28342988 |
2017 |
|
Influenza A
|
0.020 |
Biomarker
|
disease |
BEFREE |
We now report ZMPSTE24 is a virus-specific effector that restricts enveloped RNA and DNA viruses, including influenza A, Zika, Ebola, Sindbis, vesicular stomatitis, cowpox, and vaccinia, but not murine leukemia or adenovirus.
|
28246125 |
2017 |
|
Influenza
|
0.010 |
Biomarker
|
disease |
BEFREE |
In vivo studies demonstrate ZMPSTE24-deficient mice display higher viral burdens, enhanced cytokine production, and increased mortality after influenza infection.
|
28246125 |
2017 |
|
leukemia
|
0.010 |
Biomarker
|
disease |
BEFREE |
We now report ZMPSTE24 is a virus-specific effector that restricts enveloped RNA and DNA viruses, including influenza A, Zika, Ebola, Sindbis, vesicular stomatitis, cowpox, and vaccinia, but not murine leukemia or adenovirus.
|
28246125 |
2017 |
|
Childhood Leukemia
|
0.010 |
Biomarker
|
disease |
BEFREE |
We now report ZMPSTE24 is a virus-specific effector that restricts enveloped RNA and DNA viruses, including influenza A, Zika, Ebola, Sindbis, vesicular stomatitis, cowpox, and vaccinia, but not murine leukemia or adenovirus.
|
28246125 |
2017 |
|
Lipodystrophy
|
0.460 |
GeneticVariation
|
disease |
BEFREE |
Those findings were intriguing but also perplexing because many of the LMNA missense mutations associated with lipodystrophy are located in sequences distant from the sequences required for the farnesylation of prelamin A and ZMPSTE24-mediated conversion of prelamin A to mature lamin A.
|
27841971 |
2016 |
|
Fenestration (morphologic abnormality)
|
0.010 |
Biomarker
|
disease |
BEFREE |
Compared to previous lower resolution structures, the enhanced resolution provides: (1) a detailed view of the active site of ZMPSTE24, including water coordinating the catalytic zinc; (2) enhanced visualization of fenestrations providing access from the exterior to the interior cavity of the protein; (3) a view of the C-terminus extending away from the main body of the protein; (4) localization of ordered lipid and detergent molecules at internal and external surfaces and also projecting through fenestrations; (5) identification of water molecules associated with the surface of the internal cavity.
|
27774687 |
2017 |
|
Colorectal Carcinoma
|
0.010 |
AlteredExpression
|
disease |
BEFREE |
Downregulation of ZMPSTE24 protein expression was found in 16.9% of CRCs and 8.9% of GCs by immunohistochemistry.
|
27729169 |
2016 |