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Evidence for a role of sphingosine-1 phosphate in cardiovascular remodelling in Fabry disease.
AIMS A hallmark of Fabry disease is the concomitant development of left-ventricular hypertrophy and arterial intima-media thickening, the pathogenesis of which is thought to be related to theExpand
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Increased carotid intima-media thickness in the absence of atherosclerotic plaques in an adult population with Fabry disease.
AIM Fabry disease is considered primarily as a progressive small vessel disease, with ischaemic degenerative lesions involving the kidneys, brain and heart. Macrovascular involvement in male patientsExpand
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Dibasic cleavage site is required for sorting to the regulated secretory pathway for both pro‐ and neuropeptide Y
To investigate the signals governing routing of biologically active peptides to the regulated secretory pathway, we have expressed mutated and non‐mutated proneuropeptide Y (ProNPY) inExpand
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Rapid Site-Directed Mutagenesis Using Two-PCR-Generated DNA Fragments Reproducing the Plasmid Template
We describe a new rapid and efficient polymerase chain reaction (PCR)-based site-directed mutagenesis method. This procedure is effective with any plasmid and it employs four oligonucleotide primers.Expand
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The somatostatin-28(1-12)-NPAMAP sequence: an essential helical-promoting motif governing prosomatostatin processing at mono- and dibasic sites.
Proline residues, known to have special structural properties, induce particular conformations which participate in some biological functions. Two prolines (Pro(-9), Pro(-5)) located near theExpand
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Aortic remodelling in Fabry disease.
AIMS To evaluate thoracic aortic dilation in patients with Fabry disease (FD). METHODS AND RESULTS A cohort of 106 patients with FD (52 males; 54 females) from three European centres were studied.Expand
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Favourable side-chain orientation of cleavage site dibasic residues of prohormone in proteolytic processing by prohormone convertase 1/3.
Previous studies using selectively modified pro-ocytocin/neurophysin substrate analogues and the purified metalloprotease, pro-ocytocin/neurophysin convertase (magnolysin; EC 3.4 24.62), have shownExpand
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Measurement of plasma endothelin-1 in experimental hypertension and in healthy subjects.
BACKGROUND Endothelin-1 is an endothelium-derived potent vasoconstrictor peptide of 21 amino acids. To establish reference values in different models of hypertension and in human subjects an assayExpand
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Abnormalities of peptide metabolism in Alzheimer disease.
The steady-state level of peptide hormones represents a balance between their biosynthesis and proteolytic processing by convertases and their catabolism by proteolytic enzymes. Low levels ofExpand
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Clearance of amyloid-beta peptide by neuronal and non-neuronal cells: proteolytic degradation by secreted and membrane associated proteases.
Deposition of amyloid-beta peptide (Abeta) in the brain is an early and invariant feature of all forms of Alzheimer's disease (AD). As for all proteins or peptides, the steady-state level of AbetaExpand
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