Rod Components Research Articles

Percussion capacitor discharge welding with a magnetic-pulsed drive

Welded joints of the body component-rod type are used widely in shipbuilding, car industry, engineering and construction. Various types of hardware are used as rod components: bolts, sleeves, pins ...

Rod and cone components in the electroretinogram of the horse.

Summary Electroretinography was carried out on 10 ponies. The conditions of light preadaptation were varied in order to make a dynamic study of the equine retina. During dark adaptation it was found that after a high level of light preadaptation the gain in sensitivity of the retina was slow and poor. In the initial period of dark adaptation the b-wave was duplicated (photopic-b/scotopic-b). Determination of critical fusion frequency, amplitude-intensity curve, peak latencies of b-wave at t0 and t30 and ERG after strong light preadaptation, revealed a clear functional duality of the horse retina i. e. the retina contains functional rods and cones. This means that the histological and functional basis for possible color vision is present in the equine retina. Zusammenfassung Stäbchen- und Zapfenanteile des Pferdeelektroretinogrammes Bei 10 Ponys wurden Elektroretinogramme registriert. Zwecks Untersuchung der Dynamik der Equiden-Retina wurden die Bedingungen der Lichtpräadaptation variiert. Nach einer Präadaptation auf sehr helles Licht war die Empfindlichkeitszunahme während der Dunkeladaptation langsam und gering. Zu Beginn der Dunkeladaptation war die b-Welle verdoppelt (photopisches-b/skotopisches-b). Die Bestimmung der kritischen Verschmelzungsfrequenz der Amplituden-Intensitätskurve, der Spitzen-Latenz der b-Welle zur Zeit t0 und t30 (min) sowie des Elektroretinogrammes nach starker Licht-Präadaptation ließ klar erkennen, daß die Retina des Pferdes eine funktionelle Dualität aufweist, d. h. funktionelle Stäbchen und Zapfen enthält. Die Pferderetina besitzt somit die histologischen und funktionellen Merkmale für Farbensehen. Résumé Portions des bâtonnets et des cônes dans l'électrorétinogramme du cheval On a enregistré l'électrorétinogramme chez 10 poneys. Les conditions de l'adaptation à la lumière ont varié dans le but de rechercher la dynamique de la rétine des équidés. Après une préadaptation à une lumière très claire, l'augmentation de la sensibilité durant l'adaptation à l'obscurité fut lente et faible. L'onde b a doublé au début de l'adaptation à l'obscurité (photopique-b/skotopique-b) La détermination de la fréquence de fusion critique de la courbe amplitude-intensité, du pique de latence de l'onde b au temps t0 et t30 (min) et de l'électrorétinogramme après une préadaptation à une lumière forte a permis de constater que la rétine du cheval présente une dualité fonctionelle, à savoir qu'elle possède des bâtonnets et des cônes fonctionnels. La rétine du cheval possède ainsi les caractères histologiques et fonctionnels d'une vision colorée. Resumen Fracciones de bastoncillos y conos en el electrorretinograma del caballo En 10 ponys se registraron electrorretinogramas. Con el fin de analizar la dinámica de la retina de los équidos, se variaron las condiciones de preadaptación a la luz. Tras la preadaptación a luz muy clara era muy lento y escaso el aumento de la sensitividad durante la adaptación a la obscuridad. En el periodo inicial de la adaptación a la obscuridad, se hallaba duplicada la onda b (fotópica b / escotópica b). La determinación de la frecuencia crítica de fusión, de la curva de intensidad de amplitud, de latencias cimas de la onda b en los tiempos t0 y t30 (min) así como el electrorretinograma tras preadaptación a luz potente reveló que la retina del caballo ostenta una dualidad funcional clara, es decir contiene bastoncillos y conos funcionales. Esto significa que la retina del caballo dispone de las características histológicas y funcionales para una visión posible de colores.

Innate immune detection of the type III secretion apparatus through the NLRC4 inflammasome

The mammalian innate immune system uses Toll-like receptors (TLRs) and Nod-LRRs (NLRs) to detect microbial components during infection. Often these molecules work in concert; for example, the TLRs can stimulate the production of the proforms of the cytokines IL-1beta and IL-18, whereas certain NLRs trigger their subsequent proteolytic processing via caspase 1. Gram-negative bacteria use type III secretion systems (T3SS) to deliver virulence factors to the cytosol of host cells, where they modulate cell physiology to favor the pathogen. We show here that NLRC4/Ipaf detects the basal body rod component of the T3SS apparatus (rod protein) from S. typhimurium (PrgJ), Burkholderia pseudomallei (BsaK), Escherichia coli (EprJ and EscI), Shigella flexneri (MxiI), and Pseudomonas aeruginosa (PscI). These rod proteins share a sequence motif that is essential for detection by NLRC4; a similar motif is found in flagellin that is also detected by NLRC4. S. typhimurium has two T3SS: Salmonella pathogenicity island-1 (SPI1), which encodes the rod protein PrgJ, and SPI2, which encodes the rod protein SsaI. Although PrgJ is detected by NLRC4, SsaI is not, and this evasion is required for virulence in mice. The detection of a conserved component of the T3SS apparatus enables innate immune responses to virulent bacteria through a single pathway, a strategy that is divergent from that used by plants in which multiple NB-LRR proteins are used to detect T3SS effectors or their effects on cells. Furthermore, the specific detection of the virulence machinery permits the discrimination between pathogenic and nonpathogenic bacteria.

HumanRPE65Gene Therapy for Leber Congenital Amaurosis: Persistence of Early Visual Improvements and Safety at 1 Year

Human gene therapy with rAAV2-vector was performed for the RPE65 form of childhood blindness called Leber congenital amaurosis. In three contemporaneous studies by independent groups, the procedure was deemed safe and there was evidence of visual gain in the short term. At 12 months after treatment, our young adult subjects remained healthy and without vector-related serious adverse events. Results of immunological assays to identify reaction to AAV serotype 2 capsid were unchanged from baseline measurements. Results of clinical eye examinations of study and control eyes, including visual acuities and central retinal structure by in vivo microscopy, were not different from those at the 3-month time point. The remarkable improvements in visual sensitivity we reported by 3 months were unchanged at 12 months. The retinal extent and magnitude of rod and cone components of the visual sensitivity between 3 and 12 months were also the same. The safety and efficacy of human retinal gene transfer with rAAV2-RPE65 vector extends to at least 1 year posttreatment.

NLRC4 is an innate immune detector of the bacterial type III secretion apparatus (135.72)

Abstract The mammalian innate immune system uses Toll-like receptors (TLRs) and Nod-LRRs (NLRs) to detect microbial components during infection. Often these molecules work in concert; for example, the TLRs can stimulate the production of the pro-forms of the cytokines IL-1β and IL-18, whereas certain NLRs trigger their subsequent proteolytic processing via caspase 1. Gram negative bacteria utilize type III secretion systems (T3SS) to deliver virulence factors to the cytosol of host cells, where they modulate cell physiology to favour the pathogen. We show here that NLRC4/Ipaf detects the basal body rod component of the T3SS apparatus (rod protein) from S. typhimurium (PrgJ), Escherichia coli (EprJ and EscI), Burkholderia pseudomallei (BsaK), and Shigella flexneri (MxiI). These rod proteins share a sequence motif that is essential for detection by NLRC4; a similar motif is found in flagellin which is also detected by NLRC4. S. typhimurium has two T3SS: Salmonella pathogenicity island-1 (SPI-1), which encodes the rod protein PrgJ, and SPI-2, which encodes the rod protein SsaI. While PrgJ is detected by NLRC4, SsaI is not, and this evasion is required for virulence in mice. Our results indicate that macrophages respond to the activity of T3SS, not by sensing the specific translocated virulence factors, but rather by detecting the translocation apparatus that is conserved between pathogens. This reduces the number of host detectors required, a survival strategy that is divergent from that used by plants, where translocated effectors are detected on a gene for gene basis. Furthermore, the specific detection of the virulence machinery permits the discrimination between pathogenic and non pathogenic bacteria.

A study of the human rod and cone electroretinogram a-wave component

The study of the electrical response of the retina to a luminous stimulus is one of the mainfields of research in ocular electrophysiology. The features of the first component (a-wave)of the retinal response reflect the functional integrity of the two populations ofphotoreceptors: rods and cones. We fit the a-wave for pathological subjects with functionsthat account for possible mechanisms governing the kinetics of the photoreceptors. Thepaper extends a previous analysis, carried out for normal subjects, in which bothpopulations are active, to patients affected by two particular diseases that reduce theworking populations to only one. The pathologies investigated are Achromatopsia, acone disease, and Congenital Stationary Night Blindness, a rod problem. Wepresent evidence that the analysis of a pathological a-wave can be employed toquantitatively measure either cone or rod activities and to test hypotheses abouttheir responses. The results show that the photoreceptoral responses differ in thetwo cases and functions implying a different number of photocascade stages arenecessary to achieve a correct modeling of the early phototransduction process.Numerical values of the parameters characterizing the best-fit functions are given anddiscussed.

Combining RNA Interference Mutants and Comparative Proteomics to Identify Protein Components and Dependences in a Eukaryotic Flagellum

Eukaryotic flagella from organisms such as Trypanosoma brucei can be isolated and their protein components identified by mass spectrometry. Here we used a comparative approach utilizing two-dimensional difference gel electrophoresis and isobaric tags for relative and absolute quantitation to reveal protein components of flagellar structures via ablation by inducible RNA interference mutation. By this approach we identified 20 novel components of the paraflagellar rod (PFR). Using epitope tagging we validated a subset of these as being present within the PFR by immunofluorescence. Bioinformatic analysis of the PFR cohort reveals a likely calcium/calmodulin regulatory/signaling linkage between some components. We extended the RNA interference mutant/comparative proteomic analysis to individual novel components of our PFR proteome, showing that the approach has the power to reveal dependences between subgroups within the cohort.

A coarse-grained simulation study of mesophase formation in a series of rod–coil multiblock copolymers

The mesophase behaviour of a rod-coil multiblock copolymer is assessed by means of a new soft-core simulation model, which is suitable for the simulation of combinations of isotropic and anisotropic particles. The simulations demonstrate the presence of isotropic melt, micelles, lamellar, nematic and gyroid phases, with ordered phases able to grow spontaneously from the isotropic melt. The influence of increasing the length of the rigid rod-component of the polymer is studied, with mesophase stability enhanced by increases in rod length. Increased nematic phase stability is demonstrated also as the length of rod component is increased. For longer rods (smaller fraction of coils) the models show evidence of metastable chevron-like structures that initially form on cooling from the polymer melt. These are eventually lost in favour of true lamellar ordering over long annealing runs. The structure of molecules within the phases formed is also assessed. Chains in the lamellar phase are shown to produce both bridging and loop behaviour, with the latter preferred slightly. Simulations at a low occupied volume fraction, corresponding to self-assembly in solution, demonstrate the formation of a structurally ordered nanowire.

Life History of Cones in the Rhodopsin-Mutant P23H-3 Rat: Evidence of Long-term Survival

To follow the status of cones over the life of the P23H-3 transgenic rat, while the rod population is depleted. P23H-3 heterozygous and Sprague-Dawley (SD) control rats were raised in dim, cyclic light from postnatal day (P)10 to P540. Retinas were examined for cone density, cone outer segment (OS) length, cone axon and soma morphology, and the amplitude of rod and cone components of the electroretinogram (ERG) were determined. In the P23H-3 retina, cone density followed a developmental pattern, increasing from P10 until P20, declining during early adult life (to P150), then steadying at levels found in the SD retina until P540. Cone OSs elongated to P30 and then slowly shortened during late adulthood; at P350 and P540, cone OSs were significantly shorter than in the background SD strain. Cone axons shortened slowly throughout adult life as the outer nuclear layer thinned. The rod a-wave declined steadily in the P23H-3 retina from P10, falling below amplitudes seen in the SD strain from early life. By contrast, the cone b-wave maintained amplitude at SD levels, until P380. Despite the ongoing loss of rod function and numbers, cone numbers in the P23H-3 retina were maintained at levels found in the SD rat to the oldest age examined, and cone function and OS morphology were maintained for approximately 1 year, indicating a long period of cone independence. The long period of cone survival creates an opportunity to induce self-repair, if the stress causing their dysfunction can be reduced.

Generation of a Transgenic Rabbit Model of Retinal Degeneration

To generate a transgenic (Tg) rabbit model of retinal degeneration and to characterize the pattern of degeneration by using histology and electrophysiology. Rhodopsin Pro347Leu Tg rabbits were generated by BAC transgenesis. Tg rabbits were identified by Southern blot analysis, and the expression levels were measured by quantitative RT-PCR. Retinal histology was examined by light and electron microscopy and immunohistochemistry. Retinal function was assessed by full-field electroretinograms (ERGs). Six lines of Tg rabbits were generated, and two lines with higher levels of expression showed rod-dominant progressive retinal degeneration. Retinal histology indicated a marked regional variation in the loss of photoreceptors with the central retina more severely affected than the peripheral retina. The characteristics of the ERGs of transgenic rabbits indicated that the rod components of the ERGs were reduced to only 5% by 48 weeks, whereas the cone components remained at 35% in the wild-type at the same time point. The retinal ultrastructure of Tg rabbits showed a large number of small vesicles that accumulated in the extracellular space of the photoreceptors. To the best of the authors' knowledge, this is the first rabbit model of progressive retinal degeneration. Because rabbits have large eyes and are easy to handle and breed, they will provide a useful animal model for the study of the pathophysiology of and new treatments for retinal degeneration.

Self-assembly of rod–coil block copolymers

Rod–coil block copolymers are an increasingly important class of molecules for the self-assembly of functional polymer systems, many of which have rodlike chain conformations due to rigid secondary structures, extended π-conjugation, or aromatic groups along the polymer backbone. Examples of these polymers are helical proteins, polyisocyanates, main-chain semiconducting polymers, and aromatic polyesters, polyamides, or polyimines. Many hindered or liquid crystalline systems self-assembled from block copolymers, including dendronized polymers and mesogen-jacketed liquid crystalline polymers, also form rod–coil block copolymers. In these cases, steric crowding of the bulky or mesogenic side chains causes the polymer backbone to become quasi-linear. The incorporation of rigid rod polymers into the block copolymers results in extremely rich self-assembly behavior that differs markedly from that of traditional block copolymers due to the interplay between microphase separation of the rod and coil components and liquid crystalline alignment. The combination of these effects results in novel structures both in solution and melts. This review discusses in detail the self-assembly and thermodynamics of rod–coil diblock and triblock copolymers. After summarizing the applications of these materials, their aggregation and gelation in solution is discussed. Our knowledge of their bulk phase behavior is thoroughly reviewed both from the experimental and theoretical perspectives, and the self-assembly of these materials in thin film geometries that are critical to many applications in organic electronics and functional surface patterning is treated. Finally, the outlook for the future of these systems is summarized along with current knowledge gaps and exciting areas for the advancement of the field.

A New Method for Entero-Enteral Anastomosis

Background: Hand-sewn enteral anastomoses are time-consuming at laparotomy and laparoscopy. During NOTES procedures they are extremely difficult. Stapled anastomoses require a two centimeter enterotomy in either bowel limb; this remaining defect must be either stapled or sewn closed. A new method for forming enteroenteral anastomoses (EEA) was developed and tested in bench, non-survival and survival pig models. This method allows EEAs to be formed through incisions 2 mm in diameter. If flexible access is available in one lumen, then only one incision needs to be made. This can be easily performed at open surgery, laparoscopy and NOTES. Methods: A hollow ferrous tube was tapered for insertion over a flexible guidewire. It featured ‘hinges’ to allow shape change during the anastomosis formation. This metal rod (component A) compressed the intervening small bowel tissue against a series of cylindrical hollow neodymium iron boron alloy magnets (component B), that were placed in the other small bowel limb. In NOTES procedures, using a double channel gastroscope, the two limbs to be anastomosed were held together with a suture. Tissue was grasped with forceps and a needle-knife incision allowed a nitinol 0.00035 inch diameter guidewire to be passed into the lumen. Component A was pushed into the lumen using a 7 French plastic pushing catheter. Using a similar method, a guidewire was inserted into the other small bowel limb and the cylindrical magnets were pushed into the lumen, where they locked together on the ante-mesenteric border of the small bowel, compressing the intervening tissue. The length of the anastomosis could be varied by extending the length of the metal cylinder and increasing the number of magnets. This formed a compression anastomosis. The small enteral openings were closed with TAS sutures, clips or 3-0 vicryl sutures. Results: At open and laparoscopic surgery an EEA was created with this technique. Three weeks post-surgery, the anastomoses were identified at autopsy examination. This was completed in 5 pigs, all of whom survived without complications. The autopsy examination revealed no peritoneal soiling, and well- healed anastomotic sites. The anastomoses were patent in all pigs. By NOTES, the EEA was also created using the two components with an anastomosis being formed uneventfully. Conclusion: A new method for enteroenteral anastomosis was developed and tested. The method has advantages over sutured or stapled anastomoses. The enterotomy made is very small. The method is much easier than a traditional anastomosis if a NOTES procedure is being done. The new device was trialed in non-survival and survival studies.

Self-assembly of rod-coil-rod ABA-type triblock copolymers

Self-assembled behavior of symmetric ABA rod-coil-rod triblock copolymer melts is studied by applying self-consistent-field lattice techniques in three-dimensional space. The phase diagram is constructed to understand the effects of the chain architecture on the self-assembled behavior. Four stable structures are observed for the ABA rod-coil-rod triblock, i.e., spherelike, lamellar, gyroidlike, and cylindrical structures. Different from AB rod-coil diblock and BAB coil-rod-coil triblock copolymers, the lamellar structure observed in ABA rod-coil-rod triblock copolymer melts is not stable for high volume fraction of the rod component (f(rod)=0.8), which is attributed to the intramolecular interactions between the two rod blocks of the polymer chain. When 0.3<f(rod)<0.7, the intramolecular interactions between the two rigid blocks of the polymer chain are decrease, which results in the occurrence of some interesting metastable mixed structures. These structures have not been observed in polymers containing only one rod block, such as a lamellar-alt-lamellar structure. The results are expected to provide guidance for the design of microstructures in experiments.

Supramolecule-Regulated Photophysics of Oligo(p-phenyleneethynylene)-Based Rod–Coil Block Copolymers: Effect of Molecular Architecture

Three new topology-varied rod-coil block copolymers, comprising the same oligo(p-phenyleneethynylene) (OPE) rod components and the same coil components, were synthesized by atom-transfer radical polymerization. Their photophysical properties were systematically studied and compared in consideration of their solid-state structures and self-assembly abilities. These copolymers have similar intrinsic photophysical properties to the OPE rods, as reflected in dilute solution. However, their photophysical properties in the solid state are manipulated to be dissimilar by supramolecular organization. Wide-angle X-ray diffraction (WAXD) and atomic force microscopy (AFM) data demonstrate that these copolymers possess different self-assembly abilities due to the molecular-architecture-dependent pi-pi interactions of the rods. Hence, the aggregates in the solid state are formed with a different mechanism for these copolymers, bringing about the discrepancy in the solid-state luminescent properties.

The mechanism of outer membrane penetration by the eubacterial flagellum and implications for spirochete evolution

The rod component of the bacterial flagellum polymerizes from the inner membrane across the periplasmic space and stops at a length of 25 nm at the outer membrane. Bushing structures, the P- and L-rings, polymerize around the distal rod and form a pore in the outer membrane. The flagellar hook structure is then added to the distal rod growing outside the cell. Hook polymerization stops after the rod-hook structure reaches approximately 80 nm in length. This study describes mutants in the distal rod protein FlgG that fail to terminate rod growth. The mutant FlgG subunits continue to polymerize close to the length of the normal rod-hook structure of 80 nm. These filamentous rod structures have multiple P-rings and fail to form the L-ring pore at the outer membrane. The flagella grow within the periplasm similar to spirochete flagella. This provides a simple method to evolve intracellular flagella as in spirochetes. The mechanism that couples rod growth termination to the ring assembly and outer membrane penetration exemplifies the importance of stopping points in the construction of a complex macromolecular machine that facilitate efficient coupling to the next step in the assembly pathway.

Study of self-assembly of symmetric coil-rod-coil ABA-type triblock copolymers by self-consistent field lattice method

The self-assembly of symmetric coil-rod-coil ABA-type triblock copolymer melts is studied by applying self-consistent field lattice techniques in a three-dimensional space. The self-assembled ordered structures differ significantly with the variation of the volume fraction of the rod component, which include lamellar, wave lamellar, gyroid, perforated lamellar, cylindrical, and spherical-like phases. To understand the physical essence of these phases and the regimes of occurrence, we construct the phase diagram, which matches qualitatively with the existing experimental results. Compared with the coil-rod AB diblock copolymer, our results revealed that the interfacial grafting density of the separating rod and coil segments shows important influence on the self-assembly behaviors of symmetric coil-rod-coil ABA triblock copolymer melts. We found that the order-disorder transition point changes from f(rod)=0.5 for AB diblock copolymers to f(rod)=0.6 for ABA triblock copolymers. Our results also show that the spherical-like and cylindrical phases occupy most of the region in the phase diagram, and the lamellar phase is found stable only at the high volume fraction of the rod.

Posttranscriptional Control of the Salmonella enterica Flagellar Hook Protein FlgE

Previous work suggested that the FlgE (flagellar hook subunit) protein in Salmonella enterica serovar Typhimurium was posttranscriptionally regulated in response to the stage of flagellar assembly. Specifically, the FlgE protein could be detected in flagellar mutants defective at the stages of assembly before or after rod assembly but not in rod assembly mutants, yet flgE mRNA levels were unaffected. To elucidate posttranscriptional mechanisms involved in the coupling of flgE gene expression to hook assembly, the RNA sequences at the 5' and 3' ends of the flgE-containing mRNA processed from the large flgBCDEFGHIJKL operon were determined by rapid amplification of cDNA ends, and secretion of the FlgE protein in different flagellar assembly mutant strains was analyzed. The sequences 5' and 3' of the flgE gene where RNA processing occurred was within 15 bases upstream of the flgD stop codon and at bases 145 to 147 downstream of the flgF start codon, respectively. The ribosome binding site of the flgD gene was found to be inhibitory to flgE translation in strains deleted for the upstream flgD gene, unless the region 15 bases upstream of the flgD stop codon was present. Secretion of FlgE into the periplasm was monitored using beta-lactamase (Bla) fusions as a periplasm-specific reporter, which conferred resistance to ampicillin when FlgE-Bla was secreted into the periplasm. Using this assay, we found that the effect of rod assembly mutants on FlgE levels was due to FlgE turnover in the periplasm and that the FliE rod component protein was required for efficient FlgE-Bla secretion.

Recovery of rod-mediated a-wave during light-adaptation in mGluR6-deficient mice

The purpose of this study was to compare the a-waves of mGluR6-deficient mice (KO) to that of wild-type mice (WT), and to determine whether the light-adapted electroretinogram of the KO mice originate exclusively from cones. Dark-adapted a-waves were recorded under the same conditions from both types of mice. With a 96-cd/m 2 background, the a-wave from both types of mice showed a rapid recovery over a 50-min period. The analysis of the a-waves in KO mice indicated that the recovery was determined mainly by the rod component. The light-adapted b-wave of WT mice showed no corresponding recovery. We conclude that rod contribution must be considered in the analyses of the light-adapted a-waves of KO mice.

A big lift for the aerospace industry: Peening technology enables planes to fly lighter and longer

The daring knights of yore somehow stumbled upon peening as a method for saving their hides. Pounding a sword blade with a rounded hammer extended the life of the weapon and, no doubt, the life of its owner as well. To this day, peening has far-reaching safety consequences as manufacturers strive to produce stronger, lighter products, particularly in the fields of automobile and aircraft production. Automobile manufacturers often peen defensively, meaning they fall back on the process to strengthen a spring, gear, connecting rod, anti-roll bar, or other component that fails prematurely. Because its products travel at higher altitudes, the aerospace industry takes a more proactive approach through the development of stringent peening specifications, in which adherence must frequently be documented. fying inspection reports related to the peening of parts used in 1,000 fighter planes built by McDonnell Douglas Corp. As a consequence, the Pentagon delayed acceptance ofF-15 Eagles, F/A-18 Hornets, and AV-88 Harriers, as well as Harpoon and Stand-off Land-attack missiles. On civilian aircraft, off-spec or nonexistent shot peening has been cited as the cause of failures on parts ranging from engine shafts to landing gears. Recognizing the impor-

PIII and Derived PII Analysis in a Patient with Retinal Dysfunction with Supernormal Scotopic ERG

To present electroretinographic (ERG) findings in a patient with retinal dysfunction with supernormal scotopic ERG, and to analyze rod and cone PIII components and rod inner nuclear layer (derived PII) responses. A Japanese 11-year-old girl complained of poor visual acuity. There was no parental consanguinity in her family. The corrected visual acuity was 0.7 in both eyes. No abnormal finding was observed in both fundi. The patient underwent full-field ERGs. Rod and cone a-waves were analyzed using photoreceptor models. The derived PII responses were analyzed using a technique described by Hood and Birch. In the photopic ERG, responses to single flash and 30-Hz flicker were attenuated. In the scotopic ERG, b-wave was supernormal in amplitude in response to intense flashes, but smaller than normal and markedly delayed over a lower range of flash intensities. By the PIII analysis, phototransductions (values of S) of both rod and cone were remarkably decreased. The derived PII responses for this patient were larger than the responses for normal subjects, and the onset of the PII responses in this patient are significantly delayed compared to those in normal subjects. The ophthalmological findings in this patient are consistent with previous publications of this disease. Although it has been reported that the sites of disease action were beyond the outer segment (values of S were within the normal range), our results suggest that photoreceptors could be involved in sites of disease action in at least some patients with this disease.