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Acanthosis Nigricans
Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24.
Acro-Osteolysis
A homozygous mutation in the lamin A/C gene associated with a novel syndrome of arthropathy, tendinous calcinosis, and progeroid features.
Acro-Osteolysis
A novel homozygous LMNA mutation (p.Met540Ile) causes mandibuloacral dysplasia type A.
Acro-Osteolysis
A novel syndrome of mandibular hypoplasia, deafness, and progeroid features associated with lipodystrophy, undescended testes, and male hypogonadism.
Acro-Osteolysis
Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24.
Alopecia
Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging.
Atherosclerosis
LMNA mutations resulting in lipodystrophy and HIV protease inhibitors trigger vascular smooth muscle cell senescence and calcification: Role of ZMPSTE24 downregulation.
Carcinogenesis
Mutational and expressional alterations of ZMPSTE24, DNA damage response-related gene, in gastric and colorectal cancers.
Carcinoma
Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins.
Cardiomyopathies
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Cardiomyopathy, Dilated
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Colorectal Neoplasms
Mutational and expressional alterations of ZMPSTE24, DNA damage response-related gene, in gastric and colorectal cancers.
COVID-19
ZMPSTE24 Regulates SARS-CoV-2 Spike Protein-enhanced Expression of Endothelial PAI-1.
Cowpox
ZMPSTE24 defends against influenza and other pathogenic viruses.
Cowpox
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
Dental Caries
Genetic Association of MPPED2 and ACTN2 with Dental Caries.
Genetic Diseases, Inborn
A novel homozygous LMNA mutation (p.Met540Ile) causes mandibuloacral dysplasia type A.
Glomerulosclerosis, Focal Segmental
Focal segmental glomerulosclerosis in patients with mandibuloacral dysplasia owing to ZMPSTE24 deficiency.
Hearing Loss, Sensorineural
A novel syndrome of mandibular hypoplasia, deafness, and progeroid features associated with lipodystrophy, undescended testes, and male hypogonadism.
Heart Failure
ZMPSTE24 Is Associated with Elevated Inflammation and Progerin mRNA.
Infections
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
Influenza, Human
ZMPSTE24 defends against influenza and other pathogenic viruses.
Influenza, Human
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
Joint Diseases
A homozygous mutation in the lamin A/C gene associated with a novel syndrome of arthropathy, tendinous calcinosis, and progeroid features.
Kidney Failure, Chronic
Focal segmental glomerulosclerosis in patients with mandibuloacral dysplasia owing to ZMPSTE24 deficiency.
Laminopathies
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Laminopathies
Changes at the nuclear lamina alter binding of pioneer factor Foxa2 in aged liver.
Laminopathies
HIV protease inhibitors inhibit FACE1/ZMPSTE24: a mechanism for acquired lipodystrophy in patients on highly active antiretroviral therapy?
Laminopathies
Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy.
Laminopathies
Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome.
Laminopathies
Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy.
Laminopathies
Laminopathies: multisystem dystrophy syndromes.
Laminopathies
LMNA, ZMPSTE24, and LBR are not mutated in scleroderma.
Laminopathies
Lonafarnib: First Approval.
Laminopathies
Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors.
Laminopathies
Microcephalia with mandibular and dental dysplasia in adult Zmpste24-deficient mice.
Leukemia
ZMPSTE24 defends against influenza and other pathogenic viruses.
Leukemia
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
Lipodystrophy
A novel homozygous LMNA mutation (p.Met540Ile) causes mandibuloacral dysplasia type A.
Lipodystrophy
A Potent HIV Protease Inhibitor, Darunavir, Does Not Inhibit ZMPSTE24 or Lead to an Accumulation of Farnesyl-prelamin A in Cells.
Lipodystrophy
Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging.
Lipodystrophy
Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24.
Lipodystrophy
Failure of ossification of the occipital bone in mandibuloacral dysplasia type B.
Lipodystrophy
Hallermann-Streiff Syndrome: No Evidence for a Link to Laminopathies.
Lipodystrophy
HIV-protease inhibitors block the enzymatic activity of purified Ste24p.
Lipodystrophy
Lipodystrophies: Genetic and Acquired Body Fat Disorders.
Lipodystrophy
LMNA missense mutations causing familial partial lipodystrophy do not lead to an accumulation of prelamin A.
Lipodystrophy
LMNA mutations resulting in lipodystrophy and HIV protease inhibitors trigger vascular smooth muscle cell senescence and calcification: Role of ZMPSTE24 downregulation.
Lipodystrophy
Proteomic profiling of adipose tissue from Zmpste24-/- mice, a model of lipodystrophy and premature ageing, reveals major changes in mitochondrial function and vimentin processing.
Lipodystrophy
[Primary lipodystrophies]
Lipodystrophy, Congenital Generalized
Mandibuloacral dysplasia type A-associated progeria caused by homozygous LMNA mutation in a family from Southern China.
Liver Cirrhosis, Biliary
LBR mutation and nuclear envelope defects in a patient affected with Reynolds syndrome.
Lung Injury
Preventing loss of mechanosensation by the nuclear membranes of alveolar cells reduces lung injury in mice during mechanical ventilation.
Lymphoma
Identification and chromosomal location of two human genes encoding enzymes potentially involved in proteolytic maturation of farnesylated proteins.
Metabolic Syndrome
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Metabolic Syndrome
ZMPSTE24 Regulates SARS-CoV-2 Spike Protein-enhanced Expression of Endothelial PAI-1.
Micrognathism
Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24.
Muscle Weakness
Skeletal muscle contractile function and neuromuscular performance in Zmpste24 (-/-) mice, a murine model of human progeria.
Muscle Weakness
Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect.
Muscular Atrophy
Skeletal muscle contractile function and neuromuscular performance in Zmpste24 (-/-) mice, a murine model of human progeria.
Muscular Diseases
Type B mandibuloacral dysplasia with congenital myopathy due to homozygous ZMPSTE24 missense mutation.
Neoplasms
Genetic variation in healthy oldest-old.
Neoplasms
Mutational and expressional alterations of ZMPSTE24, DNA damage response-related gene, in gastric and colorectal cancers.
Neoplasms
Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion.
Obesity, Abdominal
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Osteoporosis
Accelerated features of age-related bone loss in zmpste24 metalloproteinase-deficient mice.
Osteoporosis
Application of micro-CT assessment of 3-D bone microstructure in preclinical and clinical studies.
Perinatal Death
A homozygous ZMPSTE24 null mutation in combination with a heterozygous mutation in the LMNA gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS.
Premature Birth
Early onset mandibuloacral dysplasia due to compound heterozygous mutations in ZMPSTE24.
Progeria
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
Progeria
A homozygous ZMPSTE24 null mutation in combination with a heterozygous mutation in the LMNA gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS.
Progeria
A humanized yeast system to analyze cleavage of prelamin A by ZMPSTE24.
Progeria
Abnormal nuclear morphology is independent of longevity in a zmpste24-deficient fish model of Hutchinson-Gilford progeria syndrome (HGPS).
Progeria
Biogenesis of the Saccharomyces cerevisiae Pheromone a-Factor, from Yeast Mating to Human Disease.
Progeria
Cell autonomous and systemic factors in progeria development.
Progeria
Depleting the methyltransferase Suv39h1 improves DNA repair and extends lifespan in a progeria mouse model.
Progeria
Different prelamin A forms accumulate in human fibroblasts: a study in experimental models and progeria.
Progeria
Dysfunction of iPSC-derived endothelial cells in human Hutchinson-Gilford progeria syndrome.
Progeria
Exome sequencing and functional analysis identifies BANF1 mutation as the cause of a hereditary progeroid syndrome.
Progeria
Genetic variation in healthy oldest-old.
Progeria
Genomic Instability and DNA Damage Responses in Progeria Arising from Defective Maturation of Prelamin A.
Progeria
Histone H4 lysine 16 hypoacetylation is associated with defective DNA repair and premature senescence in Zmpste24-deficient mice.
Progeria
Human ZMPSTE24 disease mutations: residual proteolytic activity correlates with disease severity.
Progeria
Hutchinson-Gilford progeria syndrome.
Progeria
Lonafarnib: First Approval.
Progeria
Microcephalia with mandibular and dental dysplasia in adult Zmpste24-deficient mice.
Progeria
Micromanaging aging with miRNAs: New messages from the nuclear envelope.
Progeria
miR?342?5p promotes Zmpste24?deficient mouse embryonic fibroblasts proliferation by suppressing GAS2.
Progeria
Next-Generation Sequencing and Quantitative Proteomics of Hutchinson-Gilford progeria syndrome-derived cells point to a role of nucleotide metabolism in premature aging.
Progeria
Nuclear envelope alterations generate an aging-like epigenetic pattern in mice deficient in Zmpste24 metalloprotease.
Progeria
Prelamin A acts to accelerate smooth muscle cell senescence and is a novel biomarker of human vascular aging.
Progeria
Prelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion.
Progeria
Progeroid laminopathy with restrictive dermopathy-like features caused by an isodisomic LMNA mutation p.R435C.
Progeria
Requirements for Efficient Proteolytic Cleavage of Prelamin A by ZMPSTE24.
Progeria
Site specificity determinants for prelamin A cleavage by the zinc metalloprotease ZMPSTE24.
Progeria
Skeletal muscle contractile function and neuromuscular performance in Zmpste24 (-/-) mice, a murine model of human progeria.
Progeria
The structural basis of ZMPSTE24-dependent laminopathies.
Progeria
ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability.
Pulmonary Fibrosis
Accelerated aging induced by deficiency of Zmpste24 protects old mice to develop bleomycin-induced pulmonary fibrosis.
Scleroderma, Systemic
LMNA, ZMPSTE24, and LBR are not mutated in scleroderma.
ste24 endopeptidase deficiency
A Potent HIV Protease Inhibitor, Darunavir, Does Not Inhibit ZMPSTE24 or Lead to an Accumulation of Farnesyl-prelamin A in Cells.
ste24 endopeptidase deficiency
Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation.
ste24 endopeptidase deficiency
Application of micro-CT assessment of 3-D bone microstructure in preclinical and clinical studies.
ste24 endopeptidase deficiency
Deficiency in ZMPSTE24 and resulting farnesyl-prelamin A accumulation only modestly affect mouse adipose tissue stores.
ste24 endopeptidase deficiency
Focal segmental glomerulosclerosis in patients with mandibuloacral dysplasia owing to ZMPSTE24 deficiency.
ste24 endopeptidase deficiency
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice.
ste24 endopeptidase deficiency
Histone H4 lysine 16 hypoacetylation is associated with defective DNA repair and premature senescence in Zmpste24-deficient mice.
ste24 endopeptidase deficiency
HIV protease inhibitors block the zinc metalloproteinase ZMPSTE24 and lead to an accumulation of prelamin A in cells.
ste24 endopeptidase deficiency
Nuclear envelope defects cause stem cell dysfunction in premature-aging mice.
ste24 endopeptidase deficiency
Phenotypic heterogeneity of ZMPSTE24 deficiency.
ste24 endopeptidase deficiency
Progeroid syndrome patients with ZMPSTE24 deficiency could benefit when treated with rapamycin and dimethylsulfoxide.
ste24 endopeptidase deficiency
Proteomic profiling of adipose tissue from Zmpste24-/- mice, a model of lipodystrophy and premature ageing, reveals major changes in mitochondrial function and vimentin processing.
ste24 endopeptidase deficiency
Severe mandibuloacral dysplasia caused by novel compound heterozygous ZMPSTE24 mutations in two Japanese siblings.
ste24 endopeptidase deficiency
Targeting RAS-converting enzyme 1 overcomes senescence and improves progeria-like phenotypes of ZMPSTE24 deficiency.
ste24 endopeptidase deficiency
Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect.
Vaccinia
ZMPSTE24 defends against influenza and other pathogenic viruses.
Vaccinia
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
Vesicular Stomatitis
ZMPSTE24 defends against influenza and other pathogenic viruses.
Vesicular Stomatitis
ZMPSTE24 Is Downstream Effector of Interferon-Induced Transmembrane Antiviral Activity.
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Tam, A.; Nouvet, F.J.; Fujimura-Kamada, K.; Slunt, H.; Sisodia, S.S.; Michaelis, S.
Dual roles for Ste24p in yeast a-factor maturation: NH2-terminal proteolysis and COOH-terminal CAAX processing
J. Cell Biol.
142
635-649
1998
Saccharomyces cerevisiae, Homo sapiens
brenda
Schmidt, W.K.; Tam, A.; Michaelis, S.
Reconstitution of the Ste24p-dependent N-terminal proteolytic step in yeast a-factor biogenesis
J. Biol. Chem.
275
6227-6233
2000
Saccharomyces cerevisiae, Homo sapiens
brenda
Bracha, K.; Lavy, M.; Yalovsky, S.
The Arabidopsis AtSTE24 is a CAAX protease with broad substrate specificity
J. Biol. Chem.
277
29856-29864
2002
Homo sapiens (O75844), Saccharomyces cerevisiae (P47154), Saccharomyces cerevisiae, Arabidopsis thaliana (Q8RX88)
brenda
Corrigan, D.P.; Kuszczak, D.; Rusinol, A.E.; Thewke, D.P.; Hrycyna, C.A.; Michaelis, S.; Sinensky, M.S.
Prelamin A endoproteolytic processing in vitro by recombinant Zmpste24
Biochem. J.
387
129-138
2005
Homo sapiens
brenda
Moulson, C.L.; Go, G.; Gardner, J.M.; van der Wal, A.C.; Smitt, J.H.; van Hagen, J.M.; Miner, J.H.
Homozygous and compound heterozygous mutations in ZMPSTE24 cause the laminopathy restrictive dermopathy
J. Invest. Dermatol.
125
913-919
2005
Homo sapiens
brenda
Porter, S.B.; Hildebrandt, E.R.; Breevoort, S.R.; Mokry, D.Z.; Dore, T.M.; Schmidt, W.K.
Inhibition of the CaaX proteases Rce1p and Ste24p by peptidyl (acyloxy)methyl ketones
Biochim. Biophys. Acta
1773
853-862
2007
Arabidopsis thaliana, Saccharomyces cerevisiae, Homo sapiens
brenda
Smigiel, R.; Jakubiak, A.; Esteves-Vieira, V.; Szela, K.; Halon, A.; Jurek, T.; Levy, N.; De Sandre-Giovannoli, A.
Novel frameshifting mutations of the ZMPSTE24 gene in two siblings affected with restrictive dermopathy and review of the mutations described in the literature
Am. J. Med. Genet. A
152A
447-452
2010
Homo sapiens
brenda
Goulbourne, C.N.; Vaux, D.J.
HIV protease inhibitors inhibit FACE1/ZMPSTE24: a mechanism for acquired lipodystrophy in patients on highly active antiretroviral therapy?
Biochem. Soc. Trans.
38
292-296
2010
Homo sapiens (O75844), Homo sapiens
brenda
Ahmad, Z.; Phadke, S.R.; Arch, E.; Glass, J.; Agarwal, A.K.; Garg, A.
Homozygous null mutations in ZMPSTE24 in restrictive dermopathy: evidence of genetic heterogeneity
Clin. Genet.
81
158-164
2012
Homo sapiens (O75844)
brenda
Barrowman, J.; Hamblet, C.; Kane, M.; Michaelis, S.
Requirements for efficient proteolytic cleavage of prelamin A by ZMPSTE24
PLoS ONE
7
e32120
2012
Homo sapiens (O75844), Homo sapiens
brenda
Spear, E.D.; Hsu, E.T.; Nie, L.; Carpenter, E.P.; Hrycyna, C.A.; Michaelis, S.
ZMPSTE24 missense mutations that cause progeroid diseases decrease prelamin A cleavage activity and/or protein stability
Dis. Model. Mech.
11
dmm033670
2018
Homo sapiens (O75844), Homo sapiens
brenda
Clark, K.M.; Jenkins, J.L.; Fedoriw, N.; Dumont, M.E.
Human CaaX protease ZMPSTE24 expressed in yeast Structure and inhibition by HIV protease inhibitors
Protein Sci.
26
242-257
2017
Homo sapiens (O75844), Homo sapiens
brenda