• Journal of Genetic Medicine
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• v.17 no.2
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• pp.97-101
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• 2020

The combination of central nervous system abnormalities and renal impairment is a notable characteristic of Galloway-Mowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified. An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.

• The Korean Journal of Physiology and Pharmacology
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• v.4 no.6
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• pp.455-461
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• 2000

Zinc contained in the neurons of central nervous system is activity-dependently released and then attenuates NMDA (N-methyl-D-aspartate)-induced neurotoxicity while augmenting non-NMDA-induced neurodegeneration. Zinc also has been reported to produce antinociceptive action on the inflammation- and nerve injury-induced hyperalgesia in the behavioral test. In this study, we investigated the effects of zinc on the responses of dorsal horn cells to NMDA, kainate and graded electrical stimulation of C-fibers. In the majority of WDR cells (70.6%), zinc current-dependently inhibited WDR cell responses to NMDA and in the remaining cells, produced biphasic responses; excitation followed by inhibition. Zinc augmented the responses of WDR cells to iontophoretical application of kainate. The dominant effect of $Zn^{2+}$ on the responses of WDR cells to C-fiber stimulation was excitatory, but inhibition, excitation-inhibition and no change of the responses to C-fiber stimulation were induced. $Ca^{2+}-EDTA$ antagonized the excitatory or inhibitory effects of $Zn^{2+}$ on the WDR cell responses. These experimental findings suggest that $Zn^{2+}$ modulates the transmission of sensory information in the rat spinal cord.

• Journal of Acupuncture Research
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• v.19 no.4
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• pp.167-182
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• 2002

This experiment was designed to investigate the effects of electroacupuncture (EA) on chronic pains and factors that affected EA effects. The responses of wide dynamic range (WDR) cells to electrical stimulation of $A{\delta}$ & C afferent fibers were used as an index of pain in rats with chronic pains induced by intraplantar injection of complete Freund's adjuvant or peripheral nerve injury. In rats with chronic pains, low (2Hz) and high (100Hz) frequency EA stimulation applied to zusanli caused the inhibition of WDR cell responses in about 60% of rats and the inhibitory actions were dependent on the stimulus strength. EA stimulation also induced an excitation of WDR cell responses in 23.9% of rats and no effect in 15.8% of rats. However, it seemed that in normal rats compared to the rat with chronic pains, the incidence of which EA stimulation caused the excitation or no effect was high. Reversible spinalization almost completely blocked EA-induced inhibitory or excitatory effects. EA stimulation more frequently induced the excitation of WDR cell responses in lightly anesthetized (0.6%) rats and the enhanced responses of WDR cells were inhibited by EA stimulation in the rat anesthetized with 1.5% enflurane. These experimental findings suggest that in rats with chronic pain, EA stimulation inhibited WDR cell responses to slow $A{\delta}$ and C fiber stimulation and EA-induced inhibitory action was under the control of descending inhibitory system and degree of anesthesia.

• The Korean Journal of Physiology
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• v.24 no.2
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• pp.305-317
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• 1990

The present study was performed to investigate modification in the electrophysiological characteristics of cat dorsal horn cells during neurogenic inflammation induced by mustard oil. The results obtained were summarized as follows: 1) Following subcutaneous injection of mustard oil the majority of wide dynamic range (WDR) cells (10/15 units) showed enhanced responses (80%) to brush, while the responses to all types of mechanical stiumli were enhanced in 3/15 units. One cell was further activated by pinch and the another was not affected at all after induction of inflammation. 2) The sensitization of WDR cell was resulted from subcutaneous injection of mustard oil either inside or outside of the receptive field (RF), whereas the spontaneous activity increased only after mustard oil was injected inside of the RF. 3) In the animal with inflammation the responses of high threshold (HT) cell to noxious stimulus were not altered, while HT cell responded to such mechanical stimulus as pressure which was usually ineffective in normal animals. 4) After induction of inflammation, low threshold (LT) cell appeared to be converted to WDR cell, showing responses not only to brush but also to pressure and pinch. 5) The mustard oil-induced inflammation enhanced responses of WDR and HT cells to the thermal stimuli and also resulted in a pronounced after-discharge in WDR cells. 6) After subcutaneous injection of lidocaine, the increased background activity of WDR cells due to inflammation was almost completely abolished. 7) A subcutaneous injection of mustard oil inside of the RF invariably desensitized the dorsal horn cells which receive sensory inputs from the inflamed RF. From the results of Present study it was revealed that a neurogenic inflammation induced by mustard oil resulted in an enhancement of responses of cat dorsal horn cells to mechanical and thermal stimuli.

• The Korean Journal of Physiology
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• v.23 no.1
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• pp.151-167
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• 1989

The present study was undertaken to investigate modification in electrophysiological characteristics of cat dorsal horn cells resulting from carrageenin-induced inflammation. The followings were studied; 1) the time-course of changes in responses of the WDR (wide dynamic range) cell 1-3h after subcutaneous injection of carrageenin in its receptive field; 2) the responses of the same dorsal hern cells before and after induction of inflammation; 3) the effect of inflammation on the responsiveness of dorsal horn neurons to algogens (bradykinin & potassium); and 4) the effect of inflammation on the activity of WDR cell following administration of indomethacin and clonidine. Though responses of WDR neuron were increased dramatically during first 1h, the maximal enhancement was observed 3h after induction of inflammation especially by repetitive light tactile stimulus. Following carrageenin injection the majority of WDR neurons (10/15 units) showed enhanced responses to all the mechanical stimuli while in 3 cases responsiveness were intensified during activation by one tactile stimulus (brush or pressure). One cell was unaffected by inflammation and in another case the response was enhanced only to noxious stimulus. Five of 9 cells that could initially be driven by noxious stimulus were activated more strongly by same stimulus and even by tactile stimulus (pressure) following inflammation. In 2 cases neurons were sensitized only to noxious stimulus whereas in another 2 cells that did not show enhanced responses to noxious stimulus responses to light tactile stimulus (pressure) appeared after inflammation. Of 16 LT cells tested 6 responded to squeeze while 4 showed the characteristics of WDR cell following inflammation. No modification in responsiveness was recognized in 3 cells whereas response to only brush was enhanced in another 3 neurons. Following carrageenin injection responses of LT cell to bradykinin or $K^{+}$ were not altered whereas those of WOR neurons to bradykinin or $K^{+}$ were suppressed in 22.2% and 33.3% of cases, respectively. In two of 8 activity of HT cells were inhibited by bradykinin while in five of 8 responsiveness to $K^{+}$ were rather enhanced by inflammation. In the rest inflammation was ineffective. In inflammation-induced animal the receptive field of LT cell was not changed whereas those of WDR cell and HT cell were tremendously expanded. The enhanced responses of WDR neurons to mechanical stimuli resulted from inflammation were suppressed by intravenously injected indomethacin and clonidine suggesting that postaglandin is involved in inflammation-induced sensitization of these cells. The involvement of peripheral and central mechanisms in the modification in responsiveness of dorsal horn cells in the carrageenin-induced inflammation was discussed.

• The Korean Journal of Physiology
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• v.22 no.2
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• pp.245-257
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• 1988

The present study was undertaken to investigate the effect of clonidine on the response of the dorsal horn cells to intra-arterially administered bradykinin $(BK:40{\mu}g)$ and $K^+(4mg)$ in spinal cats and cats with intact spinal cord. The change in the activities of low threshold (LT), high threshold (HT) and wide dynamic range (WDR) cells induced by BK and $K^+$ were determined before and after treatment of animals with clonidine. Also studied was mechanism of inhibitory action of clonidine on the responses of dorsal horn cells to the chemical algogenics. Number of WDR cell responded to intra-arterially administered BK and $K^+$ was greater in spinal animals than in cats with intact spinal cord. Following administration of BK or $K^+$ no change was observed in the activity of LT cell whereas activity of HT cell increased invariably. The increased response of HT cell to BK and $K^+$ was markedly suppressed by clonidine. On the other hand, such inhibitory actions of clonidine were almost completely blocked by yohimbine. The majority of WDR cells were activated by $K^+$ while response of WDR cells to BK was diverse (excitatory, inhibitory or mixed). These results indicate that clonidine inhibits responses of the dorsal horn cells not only to thermal or mechanical stimulations but also to chemical algogenics, and that the inhibitory action of clonidine is generally mediated through excitation of ${\alpha}_2-adrenoreceptors$.

• Restorative Dentistry and Endodontics
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• v.27 no.6
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• pp.587-599
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• 2002

Extracellular single unit recordings were made from the ventral posteromedial thalamic (VPM) nociceptive neurons to determine mechanoreceptive field (RF) and response properties. A total of 44 VPM thalamic nociceptive neurons were isolated from rats anesthetized with urethane-chloralose. Based on responses to various mechanical stimuli including touch, pressure and pinch applied to the RF, 32 of 44 neurons were classified as nociceptive specific (NS) neuron. The other 12 neurons, classified as wide dynamic range (WDR), showed a graded response to increasingly intense stimuli, with a maximum discharge to noxious pinch. The VPM nociceptive neurons showed various spontaneous activity ranged from 0-6 Hz. They were located throughout the VPM, and had an contralateral RF including mainly intraoral (and perioral) regions. The RF size was relatively small, and very few neurons had a receptive field involving 3 trigeminal divisions. The NS neurons activated only by pressure and pinch stimuli had high mechanical thresholds compared to WDR neurons activated also by touch stimuli. The VPM nociceptive neurons were tested with suprathershold graded mechanical stimuli. Most of 21 NS and 8 WDR neurons showed a progressive increase in number of spikes as mechanical stimulus intensity was increased. In some neurons, the responses reached a peak before the highest intensity was given. Application of 5 mM $CoCl_2{\;}(10{\;}{\mu}\ell)$ solution to the trigeminal subnucleus caudalis did not produce any significant changes in the spontaneous activity, RF size, mechanical threshold, and response to suprathreshold mechanical stimuli of 9 VPM nociceptive neurons tested. 17 of 33 VPM nociceptive neurons responded to noxious heat as well as noxious mechanical stimuli applied to their RF. Application of the mustard oil, a small-fiber excitant and inflammatory irritant, to the right maxillary first molar tooth pulp induced an immediate but short-lasting neuronal discharges upto approximately 4 min in 16 of 42 VPM nociceptive neurons. These results suggest that VPM thalamic nucleus may contribute to the sensory discriminative aspect of orofacial nociception.

• Restorative Dentistry and Endodontics
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• v.24 no.4
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• pp.614-622
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• 1999

Recent studies have implicated that more rostral components of the trigeminal spinal nucleus including subnucleus oralis (Vo) in orofacial nociceptive mechanisms. Since there is only limited electrophysiological evidence, the present study was initiated to characterize the receptive field and response properties of malls nociceptive neurons in chloralose/urethan-anesthetized rats. Single neuronal activity was recorded in right subnucleus oralis, and types of nociceptive neurons classified wide dynamic range (WDR), NS (nociceptive specific) and deep nociceptive (D) and the mechanoreceptive field (RF) and response properties were determined. A total of 34 nociceptive neurons could be subdivided into 17WDR neurons, 12NS neurons and 5D neurons. Vo nociceptive neurons had RF involving maxillary and/or mandibular divisions mainly located in the intraoral and/or perioral regions. Majority of Vo nociceptive neurons showed spontaneous activity less than 1Hz. The NS and D neurons activated only by heavy pressure and/or pinch stimuli had high mechanical thresholds compared to WDR neurons activated also by tactile stimuli. Vo nociceptive neurons showed a progressive increase of response to the graded mechanical stimuli. 39% of Vo nociceptive neurons received C-fiber electrical input as well as A-fiber electrical input from their RF, and 45% of them responded to electrical stimulation of the right maxillary first molar. 41% of Vo nociceptive neurons responded to noxious heat applied to their RF, and 18% of them showed an immediate burst of discharges following MO application to the right maxillary first molar pulp. These results indicate that Vo is involved in the transmission of nociceptive information mainly coming from intraoral or perioral region including tooth pulp.

• Journal of the Institute of Convergence Signal Processing
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• v.10 no.4
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• pp.267-273
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• 2009

In this paper, we proposed a variable average operation for a WDR(Wide Dynamic Range). The previously proposed average operation [5] improves hardware efficiency and complexity by replacing divider with multiplier. However, the previously proposed method has some weak-points. For example, there are counting horizontal and vertical length, and then the multiplier selects a Mode set by the user when the lengths exactly correspond with the image's size in the Mode. To compensate some weak-points, we change a Mode selection methods as a using the image's total size. Also, we propose another feature that it can be applied to various image sizes. To get a more accurate average, we add an external compensation value. We design the variable average operation using a Verilog-HDL and confirm that the Serial Multiplier's structure is better efficiency than Split Multiplier's structure.

• Molecules and Cells
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• v.41 no.7
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• pp.676-683
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• 2018

Cilia are highly specialized antennae-like organelles that extend from the cell surface and act as cell signaling hubs. Intraflagellar transport (IFT) is a specialized form of intracellular protein trafficking that is required for the assembly and maintenance of cilia. Because cilia are so important, mutations in several IFT components lead to human disease. Thus, clarifying the molecular functions of the IFT proteins is a high priority in cilia biology. Live imaging in various species and cellular preparations has proven to be an important technique in both the discovery of IFT and the mechanisms by which it functions. Live imaging of Drosophila cilia, however, has not yet been reported. Here, we have visualized the movement of IFT in Drosophila cilia using time-lapse live imaging for the first time. We found that NOMPB-GFP (IFT88) moves according to distinct parameters depending on the ciliary segment. NOMPB-GFP moves at a similar speed in proximal and distal cilia toward the tip (${\sim}0.45{\mu}m/s$). As it returns to the ciliary base, however, NOMPB-GFP moves at ${\sim}0.12{\mu}m/s$ in distal cilia, accelerating to ${\sim}0.70{\mu}m/s$ in proximal cilia. Furthermore, while live imaging NOMPB-GFP, we observed one of the IFT proteins required for retrograde movement, Oseg4 (WDR35), is also required for anterograde movement in distal cilia. We anticipate our time-lapse live imaging analysis technique in Drosophila cilia will be a good starting point for a more sophisticated analysis of IFT and its molecular mechanisms.