Chromosome mapping of the human mitochondrial acetoacetyl-coenzyme A thiolase gene to 11q22.3----q23.1 by fluorescence in situ hybridization.
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Acetyl-CoA C-Acetyltransferase↗
Biomedical subjects
M Kano
Publications and source records attributed to M Kano.
Further analysis of mutant thiolase protein in fibroblasts from a Japanese boy with 3-ketothiolase deficiency.
We examined the mutant protein of mitochondrial acetoacetyl-CoA thiolase (mutant T2) in fibroblasts from a Japanese boy with 3-ketothiolase deficiency. The molecular size of the mutant T2 protein, determined by pulse labeling and SDS/PAGE, was intermediate between the mature subunit and the precursor of T2. To characterize the mutant T2 protein, pulse-labeling and rhodamine 6G inhibition of mitochondrial transport in fibroblasts, cell-free translation experiments, and family studies by thiolase assay, immunoblotting, and pulse-labeling were carried out. The mutant T2 was detectable as early as a 10-min pulse. The probable precursor of the mutant T2 was not detectable in either the rhodamine 6G inhibition or cell-free translation experiments. In the parents, the K+ ion dependency of acetoacetyl-CoA thiolase activity was low and the T2 bands in immunoblots were faint. It would thus appear that the parents are heterozygotes of this disease. In pulse-labeling, only a band for the mutant T2 was detected in the patient and a single band for the normal mature subunit of T2 in the father; both bands were detected in the mother. These findings suggested that the mutant T2 in the patient was inherited from the mother, and that the expression of another mutant allele of the father may be either abolished or scanty.
Acetyl-CoA C-Acetyltransferase↗
Mesenchymal hamartoma of the liver: report of an adult case and review of the literature.
A case of mesenchymal hamartoma of the liver incidentally found to be a hyperechoic mass by abdominal ultrasonography in a 62-year-old male is described. Computed tomography and magnetic resonance imaging of the liver confirmed the presence of a well-demarcated inhomogeneous mass in the left medial segment. Hepatic arteriography revealed the mass to be generally hypovascular. Histopathologic diagnosis of the resected specimen was a solid form of benign mesenchymal hamartoma, a rather rare lesion for the patient's age. Fifteen adult cases of hepatic mesenchymal hamartoma in the literature including the present case are reviewed.
Diagnosis, Differential↗
[Laparoscopic nephrectomy. Animal experiment and clinical application].
Laparoscopic nephrectomy was carried out on 6 sows in order to develop the procedures of clinical laparoscopic nephrectomy and the equipment necessary for this operation. Based on the animal experiments, it was shown that (1) retroperitoneal approach was difficult due to narrow space. (2) there must be at least 5 cm distance between each trocar and 4 or 5 trocar 1 cm in diameter were basically required. (3) The procedure consisted of incision and dissection of the peritoneum around the kidney, cutting and ligation of the ureter, dissection along the medial side of the ureter to approach the renal pedicle and clipping and cutting of the renal vessels. (4) The equipment required for this surgery must be functionally equivalent to those used in the open surgery. (5) In addition, the specifically designed equipment, such as a morcellator and an instrument to ligate renal vessels are necessary. The first clinical case of laparoscopic nephrectomy was a 34 years old man with a right non-functioning hydronephrosis due to ureteropelvic junction stricture. The surgery was successfully performed with a 110 ml blood loss. It took 7 and half hours because of abundant peri-renal fatty tissue and large extra-renal pelvis. The convalescence was uneventful. Laparoscopic nephrectomy can be applied on selected cases and the development of equipment will make the surgery more popular.
Animals↗
[A case of neurinoma arising from the lesser omentum in differentiating from primary hepatic cancer].
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Carcinoma, Hepatocellular↗
Molecular basis of 3-ketothiolase deficiency.
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Acetyl-CoA C-Acyltransferase↗
[The value and limitation of resting 201Tl reinjection after stress-redistribution imaging for the assessment of myocardial viability: comparison with wall motion improvement by revascularization].
Clinical value and limitation of resting reinjection of small dose of thallium (37 MBq) for the assessment of myocardial viability were evaluated. The results were compared with the degree of wall motion improvement by revascularization to infarcted myocardium supplied by chronic total vessels in 12 patients with old myocardial infarction. Thallium uptake was visually scored and judged as normal, reversible defect (Group 1), new fill in after reinjection (Group 2A), and no fill in even after reinjection (Group 2B). Among 53 segments with initial perfusion abnormality, 21 segments reverted to almost normal, while 32 segments remained abnormal on redistribution images. New fill in after reinjection was observed in 11 segments of 32 segments showing persisting defect on stress and delayed image (37%). Wall motion score index of Group 2A improved significantly higher than Group 2B (p less than 0.01) and almost equal to Group 1, suggesting the utility of reinjection for the assessment of tissue viability which may be underestimated by conventional imaging. But significant wall motion improvement (greater than or equal to 0.6 mean SD/chords) was observed in 6 segments (29%) of 21 segments showing neither redistribution nor fill in after reinjection. These data indicate that small dose of thallium reinjection may enhance detection of viable but jeopardized myocardium, although some underestimation of viability remained to be resolved.
Aged↗
Structure and expression of the human mitochondrial acetoacetyl-CoA thiolase-encoding gene.
To examine 3-ketothiolase deficiency at the gene level, we analyzed the structure of the human mitochondrial acetoacetyl-CoA thiolase (MAT; EC 2.3.1.9)-encoding gene (MAT). From the genomic library of a normal subject in lambda EMBL3, we isolated seven overlapping clones covering the entire length of MAT and the structural organization was determined. The gene spans approx. 27 kb and contains twelve exons interrupted by eleven introns. The 5'-flanking region of the gene lacks a conventional TATA box, but is G + C-rich and contains two CAAT boxes. Included are a putative binding site for the transcription factor, Sp1, and sequences resembling the binding sites of several other transcription factors, all features characteristic of housekeeping genes. A CAT assay revealed that a 101-bp DNA fragment immediately upstream from the cap site has promoter activity, and suggested that a DNA fragment from bp -888 to -102 probably contains a negative regulatory element(s).
Acetyl-CoA C-Acetyltransferase↗
Simple spike modulation of Purkinje cells in the cerebellar nodulus of the pigmented rabbit to optokinetic stimulation.
In immobilized pigmented rabbit anesthetized with N2O (70%) and halothane (2-4%), simple and complex spike responses of Purkinje cells in the cerebellar nodulus to large field optokinetic stimulation (OKS) was studied. About half of the cells showed significant simple spike modulation to OKS delivered to ipsilateral (25/57) or contralateral (18/31) eye. The preferred direction and the amplitude of modulation of simple spike responses did not necessarily correlate with those of complex spike responses, suggesting that the simple spike modulation is not secondary to complex spike modulation but due to modulation of mossy fiber activity to OKS.
Action Potentials↗
Pharmacological properties of two types of calcium channel in embryonic chick skeletal muscle cells in culture.
We have studied the effects of organic and inorganic Ca channel blockers on the two-component action potentials (low- and high-threshold components) in cultured chick skeletal muscle cells, which are generated by the T- and L-type voltage-dependent Ca channels (VDCCs), respectively. Nifedipine and D600 effectively blocked the high-threshold component of the action potential, whereas omega-conotoxin and phenytoin had no effect on this component. By contrast, the low-threshold component was insensitive to all of these organic Ca channel blockers. Blocking effects of polyvalent cations were observed with the following rank order of relative potency (mean apparent dissociation constant in microM): La3+ (14.7) greater than Ni2+ (20.7) greater than Cd2+ (51.2) greater than Co2+ (912) for the low-threshold component, and Cd2+ (0.7) greater than La3+ (29.2) greater than Co2+ (431) greater than Ni2+ (1241) for the high-threshold component. Taken together, these findings suggest that the pharmacological properties of the T- and L-type VDCCs in cultured chick skeletal muscle cells may differ from those in other preparations.
Action Potentials↗
Optokinetic response of simple spikes of Purkinje cells in the cerebellar flocculus and nodulus of the pigmented rabbit.
Under anesthesia with N2O (70%) and halothane (2-4%), Purkinje cell activities were extracellularly recorded in the flocculus and nodulus of immobilized pigmented rabbits. Large field (60 degrees x 60 degrees) optokinetic stimulation (OKS) was delivered to the central visual field of one eye with a constant velocity (0.1-4.0 degrees/s) at 0 degrees, 45 degrees, 90 degrees or 135 degrees to the horizontal plane of the eye. Most of the Purkinje cells in the flocculus and the nodulus showed significant simple spike modulations to OKS delivered to either eye. As a whole, the preferred directions of simple spike responses in the flocculus had the same orientation as those of complex spike responses. However, the preferred directions and amplitudes of modulation of simple spike responses did not necessarily correlate with those of complex spike responses in individual flocculus Purkinje cells. On the other hand, the preferred directions of simple and complex spike responses were not necessarily in the same orientation in the nodulus. The optimum velocity for simple spike responses was in the range 0.1-2.0 degrees/s for Purkinje cells in both the flocculus and the nodulus. The amplitude and time to peak of the simple spike responses of nodulus Purkinje cells were significantly smaller and longer, respectively, than those of flocculus Purkinje cells. In both the flocculus and the nodulus, Purkinje cells whose simple spikes preferred the horizontal orientation (H cells) and the vertical orientation (V cells) showed clustering. In particular, zonal organization was noted in the flocculus. H cells were localized in a dorso-ventral zone in the rostral one third of the flocculus, and V cells were in two distinct zones rostral and caudal to the H cell zone. The locations of H and V cells in the flocculus correspond to the H zone and V zones, respectively, determined on the basis of the preferred directions of complex spike responses to OKS. This indicates that the same subdivisions of the flocculus are supplied with optokinetic signals with the same orientation selectively through both mossy and climbing fibers, and suggest that such subdivisions of the flocculus are functional units which control horizontal and vertical components of optokinetic eye movements. The present results indicate that the flocculus and the nodulus are supplied with distinct optokinetic signals through mossy fibers and play different roles in controlling optokinetic eye movements.
Animals↗
Optokinetic response of cells in the nucleus reticularis tegmenti pontis of the pigmented rabbit.
In immobilized pigmented rabbits anesthetized with N2O (70%) and halothane (2-4%), extracellular spikes were recorded from neurons in the nucleus reticularis tegmenti pontks (NRTP) and their responses to optokinetic stimulation (OKS) were examined. OKS was delivered using constant-velocity (0.1-4.0 degrees/s) movements of a random dot pattern (60 degrees x 60 degrees) at 0 degree, 45 degrees, 90 degrees or 135 degrees to the horizon. With OKS delivered to the contralateral eye (n = 43), the preferred directions of NRTP cells were forward (F, n = 10), backward (B, n = 7), downward (D, n = 5), and the remaining cells showed no response (N, n = 21). With OKS delivered to the ipsilateral eye (n = 43), the preferred directions were F (n = 8), B (n = 8), upward (U, n = 2), D (n = 1) and N (n = 24). The majority of cells which responded to OKS (17/22 for contralateral, and 16/19 for ipsilateral OKS) preferred the horizontal orientation. The optimum velocity ranged from 0.2 to 1 degree/s. The results suggest that the NRTP cells mainly transfer horizontal optikinetic signals to the flocculus and control horizontal optokinetic eye movements.
Animals↗
Binocular interaction and signal components of optokinetic responses of climbing fiber afferents in the cerebellar flocculus and nodulus of the pigmented rabbit.
Under anesthesia with N2O (70%) and halothane (2-4%), Purkinje cells were extracellularly recorded in the flocculus and nodulus of immobilized pigmented rabbits, and complex spike responses to optokinetic stimulation (OKS) delivered simultaneously to both the ipsi- and contralateral eyes (binocular OKS) were compared with those to OKS monocularly delivered to the ipsi- or contralateral eye (monocular OKS). The complex spike responses to binocular OKS were an approximate summation of monocular responses to ipsi- and contralateral OKS. The complex spike modulation did not correlate with rhythmical slow and quick nystagmic discharges of the oculomotor nerve evoked by horizontal OKS. This suggests that complex spike responses originate largely from 'retinal slip' signals rather than from 'final motor command' signals represented in the discharges of the oculomotor nerve.
Afferent Pathways↗
Intradendritic release of calcium induced by glutamate in cerebellar Purkinje cells.
The ability of excitatory amino acids to induce increases in the intracellular Ca2+ concentration ([Ca2+]i) of cerebellar Purkinje cells was examined by digital fluorescence ratio imaging of voltage-clamped Purkinje cells dialyzed with the Ca2+ indicator fura-2. Purkinje cells responded with large inward currents accompanied by increases in dendritic [Ca2+]i when challenged with the excitatory amino acid agonists glutamate and quisqualate. The rise in [Ca2+]i was transient and reached peak values of several hundred nanomolar. The response subsisted in the absence of extracellular Ca2+, a condition that eliminates Ca2+ entry through voltage-gated Ca2+ channels, indicating that Ca2+ arose in large part from an intracellular compartment. In support of this hypothesis, only the first agonist application elicited a [Ca2+]i increase in slices maintained in Ca(2+)-free medium, as expected if the intracellular stores become depleted. These results indicate that metabotropic glutamate receptors are functional in Purkinje cells and point to glutamate as a possible modulator of [Ca2+]i in these neurons.
Animals↗
Parasympathetic denervation of rat pial vessels significantly increases infarction volume following middle cerebral artery occlusion.
Studies were undertaken in Long Evans rats to examine the hypothesis that chronic unilateral sectioning of vasodilating nerve fibers (parasympathetic and/or sensory) innervating the circle of Willis increases infarction volume following unilateral branch occlusion of the middle cerebral artery (MCA) combined with temporary (45 min) bilateral common carotid occlusion. Infarct size was measured 24 h after surgical occlusion from seven coronal slices. Infarction volume (mean +/- SD) in sham animals (group A) and surgically naive animals (group B) measured 153 +/- 43 and 131 +/- 38 mm3, respectively. After lesions of both sensory (nasociliary nerve) and parasympathetic efferents at the ethmoidal foramen (group C, combined lesion) or selective lesions of parasympathetic efferents (group D), infarction volume increased [214 +/- 47 mm3 (p less than 0.01) and 209 +/- 46 mm3 (p less than 0.05), respectively]. No increases were detected after cutting the nasociliary nerve alone (group E) or occluding the external ethmoidal artery (group F) [145 +/- 39 mm3 (p greater than 0.05) and 124 +/- 63 mm3 (p greater than 0.05), respectively]. The infarct was predominantly located within cortical gray matter and became enlarged on its superior and inferior aspects after parasympathectomy. Large infarcts were noted whether animals breathed spontaneously (all of the above) or were artificially respired or whether animals were anesthetized with xylazine and ketamine or chloral hydrate. Taken together, these studies suggest a previously unrecognized protective role for autonomic parasympathetic fibers in the pathophysiology of focal cerebral ischemia that is not shared by sensory fibers. The importance of autonomic vasodilating fibers to blood flow in ischemic brain merits further study.
Animals↗
Functional reorganization of adult cat somatosensory cortex is dependent on NMDA receptors.
We studied effects of selective blockade of N-methyl-D-aspartate (NMDA) receptors in the primary somatosensory cortex (SI) of the adult cat on reorganization of cortical maps after selective deafferentation. About two weeks after hindlimb deafferentation, cortical loci representing the 'trunk' seemingly expanded and 'migrated' into areas originally devoted to the 'hindlimb'. Selective blockade of NMDA receptors of the SI cells by continuous infusion of 2-amino-5-phosphonovalerate (APV) into the cortex disrupted such change. The APV treatment also depressed responses of SI cells to natural sensory stimulation, but left no long-lasting side effects. The results suggest that the processes of map reorganization take place within the SI and involve mechanisms dependent on NMDA-receptor-mediated activity.
2-Amino-5-phosphonovalerate↗
Serum creatine kinase activities in dogs with dirofilariasis.
Total serum creatine kinase (CK) and its isozyme activities were determined in dogs with dirofilariasis. Before heartworm removal, total CK and isozyme activities in dogs of the mild group were not different from those in dogs of the heartworm-free group. BB activity was higher in dogs of the hemoptysis group. Dogs of the ascites group displayed a mild increase in MM activity. In dogs of the caval syndrome (CS) group, total CK and MM activities were highest among the heartworm-free and heartworm-infected dogs, and MM isozyme accounted for most (75%) of total CK activity. MB and BB activities were also higher. However, there were no significant differences in CK activities between the surviving and non-surviving cases. In dogs with pulmonary heartworm disease (mild and ascites groups), MM activity correlated significantly with the number of heartworms (r = 0.45), hematocrit value (Ht, r = -0.40), serum alanine aminotransferase (ALT, r = 0.42) and lactate dehydrogenase (LDH, r = 0.46) activities, mean pulmonary arterial pressure (r = 0.64) and total pulmonary resistance (r = 0.50). In dogs with CS, MM activity did not correlate with any parameter, but BB activity correlated with the number of heartworms at the right atrium (r = 0.61), Ht (r = -0.53), ALT (r = 0.80), LDH (r = 0.73) and serum urea nitrogen (r = 0.47). At 1 week after heartworm removal, BB and MM activity decreased in dogs of the hemoptysis and ascites groups, respectively. In dogs of the CS group, total CK and MM isozyme activities decreased markedly (P less than 0.01) regardless of their prognosis.
Airway Resistance↗
[Anticoagulant in plasmapheresis].
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Anticoagulants↗