Published in Mol Cell Biol on January 01, 2006
The biology and therapeutic targeting of the proprotein convertases. Nat Rev Drug Discov (2012) 3.18
VACTERL/caudal regression/Currarino syndrome-like malformations in mice with mutation in the proprotein convertase Pcsk5. Genes Dev (2008) 1.32
In vivo functions of the proprotein convertase PC5/6 during mouse development: Gdf11 is a likely substrate. Proc Natl Acad Sci U S A (2008) 1.20
Synthetic small molecule furin inhibitors derived from 2,5-dideoxystreptamine. Proc Natl Acad Sci U S A (2006) 1.19
On the cutting edge of proprotein convertase pharmacology: from molecular concepts to clinical applications. Biomol Concepts (2011) 1.02
A mouse diversity panel approach reveals the potential for clinical kidney injury due to DB289 not predicted by classical rodent models. Toxicol Sci (2012) 1.02
Laser-capture microdissection and transcriptional profiling of the dorsomedial nucleus of the hypothalamus. J Comp Neurol (2012) 0.95
Amyloid Precursor-like Protein 2 and Sortilin Do Not Regulate the PCSK9 Convertase-mediated Low Density Lipoprotein Receptor Degradation but Interact with Each Other. J Biol Chem (2015) 0.95
PACE4 (PCSK6): another proprotein convertase link to iron homeostasis? Haematologica (2015) 0.91
Latent transforming growth factor beta-binding proteins-2 and -3 inhibit the proprotein convertase 5/6A. J Biol Chem (2011) 0.90
Disruption of proprotein convertase 1/3 (PC1/3) expression in mice causes innate immune defects and uncontrolled cytokine secretion. J Biol Chem (2012) 0.88
Modulation of protease activated receptor 1 influences human metapneumovirus disease severity in a mouse model. PLoS One (2013) 0.88
Genetic variation at the proprotein convertase subtilisin/kexin type 5 gene modulates high-density lipoprotein cholesterol levels. Circ Cardiovasc Genet (2009) 0.87
Internalization of proprotein convertase PC7 from plasma membrane is mediated by a novel motif. J Biol Chem (2012) 0.87
An activin/furin regulatory loop modulates the processing and secretion of inhibin alpha- and betaB-subunit dimers in pituitary gonadotrope cells. J Biol Chem (2008) 0.86
The proprotein convertase PC5/6 is protective against intestinal tumorigenesis: in vivo mouse model. Mol Cancer (2009) 0.86
Role of PCSK5 expression in mouse ovarian follicle development: identification of the inhibin α- and β-subunits as candidate substrates. PLoS One (2011) 0.81
Decreased PCSK9 expression in human hepatocellular carcinoma. BMC Gastroenterol (2015) 0.79
Opposite roles of furin and PC5A in N-cadherin processing. Neoplasia (2012) 0.79
RNAi-mediated silencing of prohormone convertase (PC) 5/6 expression leads to impairment in processing of cocaine- and amphetamine-regulated transcript (CART) precursor. Biochem J (2006) 0.78
Proprotein convertase subtilisin/kexin type 7 (PCSK7) is essential for the zebrafish development and bioavailability of transforming growth factor β1a (TGFβ1a). J Biol Chem (2013) 0.78
PCSK5 mutation in a patient with the VACTERL association. BMC Res Notes (2015) 0.77
Neuroinflammation-Induced Interactions between Protease-Activated Receptor 1 and Proprotein Convertases in HIV-Associated Neurocognitive Disorder. Mol Cell Biol (2015) 0.77
Phylogenetic analysis of Amphioxus genes of the proprotein convertase family, including aPC6C, a marker of epithelial fusions during embryology. Int J Biol Sci (2006) 0.75
Pcsk5 is required in the early cranio-cardiac mesoderm for heart development. BMC Dev Biol (2017) 0.75
Thrombin Activation of Protein C Requires Prior Processing by a Liver Proprotein Convertase. J Biol Chem (2017) 0.75
Spatiotemporal expression of proprotein convertase subtilisin/kexin type 5 in the development of anorectal malformations in fetal rats. J Mol Histol (2017) 0.75
A murine model of Holt-Oram syndrome defines roles of the T-box transcription factor Tbx5 in cardiogenesis and disease. Cell (2001) 5.77
The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): liver regeneration and neuronal differentiation. Proc Natl Acad Sci U S A (2003) 5.26
Statins upregulate PCSK9, the gene encoding the proprotein convertase neural apoptosis-regulated convertase-1 implicated in familial hypercholesterolemia. Arterioscler Thromb Vasc Biol (2004) 4.08
Proprotein and prohormone convertases: a family of subtilases generating diverse bioactive polypeptides. Brain Res (1999) 3.79
Decreased lipid synthesis in livers of mice with disrupted Site-1 protease gene. Proc Natl Acad Sci U S A (2001) 3.13
The family of subtilisin/kexin like pro-protein and pro-hormone convertases: divergent or shared functions. Biochimie (1994) 2.64
Defective prohormone processing and altered pancreatic islet morphology in mice lacking active SPC2. Proc Natl Acad Sci U S A (1997) 2.42
Disruption of PC1/3 expression in mice causes dwarfism and multiple neuroendocrine peptide processing defects. Proc Natl Acad Sci U S A (2002) 2.19
Failure of ventral closure and axial rotation in embryos lacking the proprotein convertase Furin. Development (1998) 1.98
In vivo functional analysis of the Hoxa-1 3' retinoic acid response element (3'RARE). Development (1997) 1.95
Regulation of membrane type-1 matrix metalloproteinase activation by proprotein convertases. Mol Biol Cell (2000) 1.90
Curbing activation: proprotein convertases in homeostasis and pathology. FASEB J (2003) 1.71
Evidence that furin is an authentic transforming growth factor-beta1-converting enzyme. Am J Pathol (2001) 1.60
SPC4/PACE4 regulates a TGFbeta signaling network during axis formation. Genes Dev (2000) 1.56
cDNA structure of the mouse and rat subtilisin/kexin-like PC5: a candidate proprotein convertase expressed in endocrine and nonendocrine cells. Proc Natl Acad Sci U S A (1993) 1.55
Limited redundancy of the proprotein convertase furin in mouse liver. J Biol Chem (2004) 1.52
Amino acids and preimplantation development of the mouse in protein-free potassium simplex optimized medium. Biol Reprod (2000) 1.52
Precursor convertases in the secretory pathway, cytosol and extracellular milieu. Essays Biochem (2002) 1.47
The isoforms of proprotein convertase PC5 are sorted to different subcellular compartments. J Cell Biol (1996) 1.36
The cysteine-rich domain of the secreted proprotein convertases PC5A and PACE4 functions as a cell surface anchor and interacts with tissue inhibitors of metalloproteinases. Mol Biol Cell (2005) 1.30
The PC6B cytoplasmic domain contains two acidic clusters that direct sorting to distinct trans-Golgi network/endosomal compartments. Mol Biol Cell (2000) 1.25
Impaired fertility in mice deficient for the testicular germ-cell protease PC4. Proc Natl Acad Sci U S A (1997) 1.24
Proprotein convertases [corrected] are responsible for proteolysis and inactivation of endothelial lipase. J Biol Chem (2005) 1.20
TACE/ADAM-17 maturation and activation of sheddase activity require proprotein convertase activity. FEBS Lett (2003) 1.15
The proprotein convertase PC5A and a metalloprotease are involved in the proteolytic processing of the neural adhesion molecule L1. J Biol Chem (2003) 1.14
Processing of alpha4 integrin by the proprotein convertases: histidine at position P6 regulates cleavage. Biochem J (2003) 1.10
Altered processing of the neurotensin/neuromedin N precursor in PC2 knock down mice: a biochemical and immunohistochemical study. J Neurochem (2002) 1.08
Implication of proprotein convertases in the processing and spread of severe acute respiratory syndrome coronavirus. Biochem Biophys Res Commun (2005) 1.08
Increased proteolytic processing of protein tyrosine phosphatase mu in confluent vascular endothelial cells: the role of PC5, a member of the subtilisin family. Biochemistry (1996) 1.04
Distinct mRNA expression of the highly homologous convertases PC5 and PACE4 in the rat brain and pituitary. J Neurosci (1995) 1.02
Bioactivation of Müllerian inhibiting substance during gonadal development by a kex2/subtilisin-like endoprotease. Proc Natl Acad Sci U S A (1996) 0.99
Structure-function analysis of the prosegment of the proprotein convertase PC5A. J Biol Chem (2002) 0.97
Peptides, enzymes and obesity: new insights from a 'dead' enzyme. Trends Biochem Sci (1999) 0.97
The metalloendopeptidase nardilysin (NRDc) is potently inhibited by heparin-binding epidermal growth factor-like growth factor (HB-EGF). Biochem J (2002) 0.97
Lefty proteins exhibit unique processing and activate the MAPK pathway. J Biol Chem (2001) 0.93
The developmental expression in the rat CNS and peripheral tissues of proteases PC5 and PACE4 mRNAs: comparison with other proprotein processing enzymes. Dev Biol (1997) 0.91
Nerve growth factor and proprotein convertases furin and PC7 in transected sciatic nerves and in nerve segments cultured in conditioned media: their presence in Schwann cells, macrophages, and smooth muscle cells. J Comp Neurol (1999) 0.89
Inhibiting uterine PC6 blocks embryo implantation: an obligatory role for a proprotein convertase in fertility. Biol Reprod (2004) 0.85
Mutations in PCSK9 cause autosomal dominant hypercholesterolemia. Nat Genet (2003) 11.67
The secretory proprotein convertase neural apoptosis-regulated convertase 1 (NARC-1): liver regeneration and neuronal differentiation. Proc Natl Acad Sci U S A (2003) 5.26
NARC-1/PCSK9 and its natural mutants: zymogen cleavage and effects on the low density lipoprotein (LDL) receptor and LDL cholesterol. J Biol Chem (2004) 4.37
Statins upregulate PCSK9, the gene encoding the proprotein convertase neural apoptosis-regulated convertase-1 implicated in familial hypercholesterolemia. Arterioscler Thromb Vasc Biol (2004) 4.08
Automated design of ligands to polypharmacological profiles. Nature (2012) 3.54
The biology and therapeutic targeting of the proprotein convertases. Nat Rev Drug Discov (2012) 3.18
The activation and physiological functions of the proprotein convertases. Int J Biochem Cell Biol (2008) 2.38
Implication of the proprotein convertases in iron homeostasis: proprotein convertase 7 sheds human transferrin receptor 1 and furin activates hepcidin. Hepatology (2013) 2.24
The cellular trafficking of the secretory proprotein convertase PCSK9 and its dependence on the LDLR. Traffic (2007) 2.09
Proprotein convertase subtilisin/kexin type 9 (PCSK9): hepatocyte-specific low-density lipoprotein receptor degradation and critical role in mouse liver regeneration. Hepatology (2008) 2.07
The proprotein convertase (PC) PCSK9 is inactivated by furin and/or PC5/6A: functional consequences of natural mutations and post-translational modifications. J Biol Chem (2006) 2.07
Proprotein convertases: lessons from knockouts. FASEB J (2006) 1.92
The proprotein convertase PCSK9 induces the degradation of low density lipoprotein receptor (LDLR) and its closest family members VLDLR and ApoER2. J Biol Chem (2007) 1.84
Rosuvastatin, proprotein convertase subtilisin/kexin type 9 concentrations, and LDL cholesterol response: the JUPITER trial. Clin Chem (2011) 1.79
Wild-type PCSK9 inhibits LDL clearance but does not affect apoB-containing lipoprotein production in mouse and cultured cells. J Lipid Res (2005) 1.79
PCSK9: a key modulator of cardiovascular health. Circ Res (2014) 1.71
Endothelium-restricted overexpression of human endothelin-1 causes vascular remodeling and endothelial dysfunction. Circulation (2004) 1.65
A new method for measurement of total plasma PCSK9: clinical applications. J Lipid Res (2010) 1.64
A serine protease inhibitor prevents endoplasmic reticulum stress-induced cleavage but not transport of the membrane-bound transcription factor ATF6. J Biol Chem (2003) 1.57
Plasma PCSK9 levels are significantly modified by statins and fibrates in humans. Lipids Health Dis (2008) 1.56
Inactivation of endothelial proprotein convertase 5/6 decreases collagen deposition in the cardiovascular system: role of fibroblast autophagy. J Mol Med (Berl) (2011) 1.51
The secretory proprotein convertases furin, PC5, and PC7 activate VEGF-C to induce tumorigenesis. J Clin Invest (2003) 1.51
Crimean-Congo hemorrhagic fever virus glycoprotein proteolytic processing by subtilase SKI-1. J Virol (2003) 1.50
The LDLR deficient mouse as a model for aortic calcification and quantification by micro-computed tomography. Atherosclerosis (2011) 1.50
Dissection of the endogenous cellular pathways of PCSK9-induced low density lipoprotein receptor degradation: evidence for an intracellular route. J Biol Chem (2009) 1.49
Precursor convertases in the secretory pathway, cytosol and extracellular milieu. Essays Biochem (2002) 1.47
Proprotein convertases in tumor progression and malignancy: novel targets in cancer therapy. Am J Pathol (2002) 1.46
Chloroquine is a potent inhibitor of SARS coronavirus infection and spread. Virol J (2005) 1.43
Biosynthesis and cellular trafficking of the convertase SKI-1/S1P: ectodomain shedding requires SKI-1 activity. J Biol Chem (2001) 1.42
The proprotein convertases are potential targets in the treatment of dyslipidemia. J Mol Med (Berl) (2007) 1.40
Regional distribution and metabolic effect of PCSK9 insLEU and R46L gene mutations and apoE genotype. Can J Cardiol (2013) 1.39
Strong induction of PCSK9 gene expression through HNF1alpha and SREBP2: mechanism for the resistance to LDL-cholesterol lowering effect of statins in dyslipidemic hamsters. J Lipid Res (2010) 1.36
Alzheimer disease Abeta production in the absence of S-palmitoylation-dependent targeting of BACE1 to lipid rafts. J Biol Chem (2008) 1.34
VACTERL/caudal regression/Currarino syndrome-like malformations in mice with mutation in the proprotein convertase Pcsk5. Genes Dev (2008) 1.32
The cysteine-rich domain of the secreted proprotein convertases PC5A and PACE4 functions as a cell surface anchor and interacts with tissue inhibitors of metalloproteinases. Mol Biol Cell (2005) 1.30
The regulated cell surface zymogen activation of the proprotein convertase PC5A directs the processing of its secretory substrates. J Biol Chem (2007) 1.30
Plasma PCSK9 levels correlate with cholesterol in men but not in women. Biochem Biophys Res Commun (2007) 1.27
Selective inhibition of proprotein convertases represses the metastatic potential of human colorectal tumor cells. J Clin Invest (2008) 1.27
The multifaceted proprotein convertases: their unique, redundant, complementary, and opposite functions. J Biol Chem (2013) 1.27
Gene inactivation of proprotein convertase subtilisin/kexin type 9 reduces atherosclerosis in mice. Circulation (2012) 1.26
Circulating proprotein convertase subtilisin/kexin 9 (PCSK9) regulates VLDLR protein and triglyceride accumulation in visceral adipose tissue. Arterioscler Thromb Vasc Biol (2011) 1.24
Antigen processing by nardilysin and thimet oligopeptidase generates cytotoxic T cell epitopes. Nat Immunol (2010) 1.24
ACAT1 gene ablation increases 24(S)-hydroxycholesterol content in the brain and ameliorates amyloid pathology in mice with AD. Proc Natl Acad Sci U S A (2010) 1.23
In vivo functions of the proprotein convertase PC5/6 during mouse development: Gdf11 is a likely substrate. Proc Natl Acad Sci U S A (2008) 1.20
Annexin A2 is a C-terminal PCSK9-binding protein that regulates endogenous low density lipoprotein receptor levels. J Biol Chem (2008) 1.20
Proprotein convertases [corrected] are responsible for proteolysis and inactivation of endothelial lipase. J Biol Chem (2005) 1.20
The proprotein convertases and their implication in sterol and/or lipid metabolism. Biol Chem (2006) 1.20
A locked nucleic acid antisense oligonucleotide (LNA) silences PCSK9 and enhances LDLR expression in vitro and in vivo. PLoS One (2010) 1.19
Involvement of matrix metalloproteinases in the adipose conversion of 3T3-L1 preadipocytes. Biochem J (2002) 1.18
Proprotein convertase subtilisin/kexin type 9 (PCSK9) can mediate degradation of the low density lipoprotein receptor-related protein 1 (LRP-1). PLoS One (2013) 1.18
In vivo evidence that furin from hepatocytes inactivates PCSK9. J Biol Chem (2010) 1.18
Atorvastatin increases intestinal expression of NPC1L1 in hyperlipidemic men. J Lipid Res (2010) 1.17
Inhibition of Chikungunya virus infection in cultured human muscle cells by furin inhibitors: impairment of the maturation of the E2 surface glycoprotein. J Biol Chem (2008) 1.14