About
28
Publications
25,271
Reads
How we measure 'reads'
A 'read' is counted each time someone views a publication summary (such as the title, abstract, and list of authors), clicks on a figure, or views or downloads the full-text. Learn more
890
Citations
Introduction
Publications
Publications (28)
Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by vegetating lesions in the folds and mouth and by the presence of autoantibodies against desmoglein 3. We describe two Caucasian patients with pemphigus vegetans, one of them presented antibodies to desmoglein 3 and 1 and the other one to desmoglein 3. Both patients also had...
Besides being present in paraneoplastic pemphigus (PNP), circulating antidesmoplakin (DP) antibodies have been found anecdotally in other bullous diseases, including pemphigus foliaceus and pemphigus vulgaris.
To verify how frequent anti-DP antibodies are in pemphigus vulgaris.
We studied 48 sera from patients with proven pemphigus vulgaris (29 muc...
Mucous membrane pemphigoid is a rare, chronic autoimmune disease characterized by subepidermal blistering and scarring, predominantly affecting mucous membranes. Ocular involvement frequently occurs and often represents the only manifestation of the disease. We describe a 62-year-old woman with a bilateral 18-month duration of conjunctival hyperaem...
The NC16A immunodominant region of the bullous pemphigoid (BP) antigen BP180 has been used to develop several enzyme-linked immunosorbent assays (ELISAs) as diagnostic tools for BP autoantibody detection.
Because BP180 autoantibody reactivity is not restricted to NC16A, we have investigated the possibility of developing an ELISA based on selected e...
Bullous pemphigoid is a subepidermal bullous disease of skin and mucosae associated with autoantibodies to BP180. To characterize the humoral response to BP180, we generated a random BP180 epitope library displayed on lambda bacteriophage. After validation of the library by epitope mapping of three BP180-specific monoclonal antibodies, 15 novel or...
Laminin-5 is the major adhesion ligand for epithelial cells. Mutations in the genes encoding laminin-5 cause junctional epidermolysis bullosa (JEB), a recessive inherited disease characterized by extensive epithelial-mesenchymal disadhesion. We describe a JEB patient compound heterozygote for two novel mutations in the gene (LAMA3) encoding the lam...
Cysteine string proteins (csps) are associated with secretory organelles in a wide range of eukaryotic cells. Functional studies of these proteins indicate that they subserve one or more vital steps in the pathway of regulated exocytosis. Here, we document the presence of csps in fully grown (stage VI) oocytes of the frog, Xenopus laevis. Both Nort...
Electrophysiological recording demonstrates that alpha-latrotoxin, a 125,000 mol. wt component of black widow spider venom, promotes high frequency quantal discharges at larval neuromuscular junctions of Drosophila. Concomitantly, fluorescence imaging of presynaptic calcium ion activity reveals that this toxin qualitatively elevates cytosolic ioniz...
Cysteine string proteins (csps) are highly conserved constituents of vertebrate and invertebrate secretory organelles. Biochemical and immunoprecipitation experiments implied that vertebrate csps were integral membrane proteins that were tethered to the outer leaflet of secretory vesicles via the fatty acyl residues of their extensively acylated cy...
A cDNA clone encoding a Xenopus cysteine string protein (Xcsp) was isolated and sequenced. The deduced primary sequence of Xcsp is very similar to other vertebrate csps with the exception of a cysteine residue that lies outside of the cysteine-string domain. This cysteine residue replaces a serine that is highly conserved among vertebrate csps, and...
A cDNA clone encoding a Xenopus cysteine string protein (Xcsp) was isolated and sequenced. The deduced primary sequence of Xcsp is very similar to other vertebrate csps with the exception of a cysteine residue that lies outside of the cysteine-string domain. This cysteine residue replaces a serine that is highly conserved among vertebrate csps, and...
The mass ipectra of several intact live and seven membrane spanning alpha helix containing intrinsic membrane proteins (IMPs) have been recorded using electrospray ioni/ation mass spectrometry (F.SI-MS). Post translational modifications including lipidation are revealed by comparison oi the measured mass with that predicted from the gene sequence....
We used tunicamycin, an inhibitor of protein fatty acylation, to examine the possibility that there is a cycle of acylation and deacylation of cysteine string proteins at nerve terminals. Using both physiological and immunoblot approaches, we obtained no evidence for a cycle of acylation and deacylation that affects these proteins. These data sugge...
The observation that is the basis of this chapter is the finding by Zinsmaier and colleagues1 that neuronal degeneration is present in organisms with mutations of the gene encoding cysteine string proteins (csps). Since there is still a very limited number of instances in which neurodegenerative disorders have been traced to alterations of single g...
Cysteine string proteins (csps) are a recently discovered class of cysteine-rich proteins. They have been shown to associate preferentially with synaptic vesicle fractions of Torpedo electric organ or rat brain where they have been implicated in events associated with transmitter secretion. However, to date there has been no information concerning...
A cDNA encoding a polypeptide of 88 amino acids was cloned following the rapid amplification of cDNA ends (RACE) procedure using mRNA isolated from the venom glands of the Mediterranean black widow spider (Latrodectus tredecimguttatus) and oligonucleotides based on the sequence of a tryptic fragment putatively from alpha-latrotoxin. Apart from a po...
A cDNA encoding a polypeptide of 88 amino acids was cloned following the rapid amplification of cDNA ends (RACE) procedure using mRNA isolated from the venom glands of the Mediterranean black widow spider (Latrodectus tredecimguttatus) and oligonucleotides based on the sequence of a tryptic fragment putatively from α-latrotoxin. Apart from a potent...
Cysteine-string proteins are relatively small, cysteine-rich components of synaptic vesicle membranes. Recent investigations demonstrated that at least 11 of the 13 cysteine residues of the Torpedo cysteine-string protein are fatty acylated. This exceptional level of fatty acylation occurs along a short stretch (less than 25 residues) of amino acid...
A brief review is presented of investigations of a novel family of synaptic vesicle proteins, the cysteine string proteins (csps). Studies of csp mutants in Drosophila reveal that csps are crucial components of the excitation-secretion machinery at nerve terminals. Current data cannot distinguish between a primary role of csps in modulating calcium...
Cysteine string proteins are novel, heavily lipidated components of synaptic vesicles. They have previously been studied in Drosophila (insect) and Torpedo (fish). To facilitate further investigation of the structure and function of these proteins in mammals, we isolated and sequenced the cDNA and conducted an initial characterization of a rat cyst...
Cysteine string proteins are relatively low mass components of synaptic vesicle membranes. Structurally, their primary sequence is distinguished by a remarkable, cysteine-rich motif. Investigations revealed an unprecedented degree of lipidation of these cysteine residues. At least 11 of the 13 cysteines of the Torpedo protein were modified, princip...
An antipeptide antiserum was raised against the C-terminal undecapeptide of a Torpedo cysteine-string protein (csp), a putative subunit or modulator of presynaptic calcium channels. This antiserum was shown to identify selectively the 27-kDa in vitro translation product of the csp cRNA both by immunoprecipitation and on immunoblots. When affinity-p...
Presynaptic calcium channels are key regulators of neurotransmitter release. Oocyte expression studies suggest that cysteine string proteins are essential subunits or modulators of these channels. Subcellular fractionation revealed that cysteine string proteins copurify with synaptic vesicles. An average vesicle had eight protein monomers with both...
A toxin that causes a massive presynaptic activation of transmitter release from nerve terminals is alpha-latrotoxin, isolated from Latrodectus tredecimguttatus spider venom. This toxin has been highly purified, utilizing as a biological assay a toxin-dependent increase in 45Ca(2+)-accumulation by PC12 cells. The purification protocol includes an i...
