PubMed Health⌕ Search

Biomedical subjects

F Kanda

Publications and source records attributed to F Kanda.

At least 37 records · Page 2Linked to original sources
PubMed1998

Severe muscle damage induced by high carbohydrate intake from elemental diet in a patient with Crohn's disease.

Crohn's disease is associated with complications in multiple organs. However, there are very few reported cases of patients with Crohn's disease with muscle symptoms and/or high serum creatine phospho-kinase (CPK) levels. We report here a patient with Crohn's disease who experienced skeletal muscle damage with extremely high serum CPK level during treatment with an elemental diet. The non-parenteral administration of large amounts of carbohydrate and limited glycogen degradation capability may be a possible causative mechanism for this elemental diet-induced muscle damage.

Adult↗
PubMed1998

Loss of serotonin-containing neurons in the raphe of patients with myotonic dystrophy: a quantitative immunohistochemical study and relation to hypersomnia.

Hypersomnia occurs frequently in patients with myotonic dystrophy (MyD). We performed a quantitative immunohistochemical study of serotonin (5-HT)-containing neurons linked to hypersomnia in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) in 8 patients with MyD, 5 of whom showed hypersomnia, and in 12 age-matched controls. The densities of 5-HT neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and controls. These data suggest that the loss of 5-HT neurons of the DRN and the SCN is associated with the presence of hypersomnia in MyD.

Aged↗
PubMed1998

Loss of catecholaminergic neurons in the medullary reticular formation in myotonic dystrophy.

OBJECTIVE: To clarify the possible relation between the extent of involvement of catecholaminergic neurons and the presence of alveolar hypoventilation in patients with myotonic dystrophy (MyD). BACKGROUND: Respiratory insufficiency has been reported frequently in MyD patients. Recent data support the hypothesis that this respiratory failure results from a primary dysfunction of the CNS. METHODS: The authors performed a quantitative immunoreactive study of tyrosine hydroxylase immunoreactive (TH+) neurons linked to hypoventilation in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN)--where the autonomic respiratory center is thought to be located--in eight MyD patients and in 10 age-matched control subjects. Alveolar hypoventilation of the central type was present in three of the MyD patients but not in the remaining MyD patients or the control subjects. RESULTS: The densities of TH+ neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in those without hypoventilation (p < 0.02, p < 0.01, and p < 0.01, respectively) and control subjects (p < 0.01, p < 0.01, and p < 0.01, respectively). CONCLUSIONS: These data suggest that the loss of TH+ neurons of the DCMN, the VCMN, and the SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.

Aged↗
PubMed1998

Fatal liver cirrhosis and esophageal variceal hemorrhage in a patient with type IIIa glycogen storage disease.

A 45-year-old woman with type IIIa glycogen storage disease (GSD IIIa) died of variceal hemorrhage secondary to liver cirrhosis. The postmortem examination disclosed increased intracellular glycogen in the liver as well as in the heart and skeletal muscle. Although most liver injuries in GSD IIIa have been considered to be non-progressive in adulthood, liver cirrhosis can be a cause of death in some patients.

Diagnosis, Differential↗
PubMed1997

Intracytoplasmic inclusion bodies of the substantia nigra in myotonic dystrophy. Immunohistochemical observations.

We recently reported a significantly higher incidence of intracytoplasmic inclusion bodies (IIBs) of the substantia nigra in patients with myotonic dystrophy (MyD) than in age-matched controls. The changes are, per se, not specific, since a small percentage of disease and normal controls also showed similar inclusions. To elucidate the pathological significance of the inclusion in MyD, we studied immunohistochemical characteristics of IIBs of the substantia nigra in eight patients with MyD. Many IIBs showed moderately intense immunoreactivity for ubiquitin, microtubule-associated protein (MAP) 1 and MAP 2. However, the IIBs did not react with any of the following: anti-neurofilament protein antibodies (Abs) (68, 160 and 200 kDa), anti-neuron-specific enolase antibody (Ab), anti-tau Ab, anti-tubulin Abs (alpha and beta), anti-paired helical filament Ab, anti-actin Ab, anti-phosphorylated epitope of neurofilaments Ab, anti-synaptophysin Ab, anti-myelin basic protein Ab, anti-actin Ab and anti-glial fibrillary acidic protein Ab. Our results suggest that IIBs of the substantia nigra in MyD are related to an alteration of neuronal cytoskeleton metabolism affecting microtubular proteins in conjunction with activation of ubiquitin proteolytic systems.

Aged↗
PubMed1997

Regulation of inducible nitric oxide synthase expression in L6 rat skeletal muscle cells.

Cytokine-stimulated expression of inducible type of nitric oxide synthase (iNOS) seems to be regulated by various signal pathways in a cell-specific manner. In this study, we examined how it was regulated in L6 rat skeletal muscle cells. In L6 cells, the combination of interleukin-1 beta and interferon-gamma induced a marked accumulation of nitrite, a stable metabolite of nitric oxide. In parallel with this reaction, iNOS mRNA expression was achieved at a maximum between 3 and 6 h, and iNOS protein was detectable at 6 h and peaked at 24 h after stimulation. Tyrosine kinase inhibitors, herbimycin A, and genistein suppressed cytokine-induced iNOS expression and nitrite production. Forskolin, an adenosine 3',5'-cyclic monophosphate-dependent protein kinase (PKA) activator, and phorbol 12-myristate 13-acetate, a protein kinase C (PKC)-activating phorbol ester, enhanced these cytokine-induced reactions. These results indicate that iNOS expression by cytokines is mediated via a protein tyrosine kinase-dependent pathway and is positively modulated by both PKA- and PKC-dependent pathways in this cell type.

Animals↗
PubMed1997

Decreased expression of full-length mRNA for cBCD541 does not correlate with spinal muscular atrophy phenotype severity.

Spinal muscular atrophy (SMA) is characterized by degeneration of spinal cord anterior horn cells and muscular atrophy and has three phenotypes based on clinical severity and age of onset. One of the responsible genes for SMA is the survival motor neuron (SMN) gene, which is homozygously absent or interrupted in more than 90% of SMA patients. The cBCD541 (BCD) gene is a highly homologous copy of the SMN gene, which has a single synonymous transition in the coding region and may compensate for the loss of the SMN gene. To evaluate the effects of the BCD gene expression on the phenotypes of SMA, we examined lymphocyte mRNA from 9 SMA patients lacking the SMN gene, 10 asymptomatic parents, and 15 control subjects. We amplified mRNA fragments containing exon 7 of the SMN or BCD genes using reverse transcription-polymerase chain reaction since the transcript lacking exon 7 encodes a putative protein with a different C-terminal end. We used glyceraldehyde-3-phosphate dehydrogenase (GAPDH) transcript as an internal control, and the relative expression level of the SMN or BCD gene was shown as the ratio of SMN or BCD transcript to GAPDH transcript (S/G ratio). The mean S/G ratios of the patients were significantly lower than that of the parents and controls. However, among the patients examined in this study, there was no relationship between the S/G ratios and phenotypes of SMA. The results showed that the BCD gene expression was not related to the phenotypes of SMA. Furthermore, there was an overlap between the S/G ratios in patients and controls. As our discrimination study showed that the S/G ratio reflected the expression of the BCD transcripts in patients and the SMN transcripts in controls, this finding suggested that the BCD gene expression per se does not compensate for the loss of the SMN gene.

Adolescent↗
PubMed1997

[Recurrent acute transverse myelopathy: an 83-year-old man with antiphospholipid syndrome].

We reported here an 83-year-old man with recurrent acute transverse myelopathy probably due to antiphospholipid syndrome (APS). The patient was admitted to a local hospital on May 8, 1996, because of hypoesthesia and muscle weakness of both legs, accompanied by dysuria. T2-weighted magnetic resonance image (MRI) showed significant swelling and increased intensity of the thoracic spinal cord between Th3 and Th7 without enhancement by contrast medium. After the treatment with steroid, both clinical symptoms and abnormal findings on MR imaging disappeared. On 24 June, however, motor paralysis of both legs and sensory loss below the chest relapsed. The lesion in the thoracic spinal cord between Th4 and Th8 reappeared on T2-weighted MRI. Blood examination disclosed the presence of a lupus anticoagulant. Repeated examinations of CSF revealed the continuous elevation of IgG. Clinical and laboratory findings indicated that immunological mechanisms related with APS might be responsible for the transverse myelopathy in this patient.

Acute Disease↗
PubMed1996

Immunohistochemical study of intracytoplasmic inclusion bodies of the thalamus in myotonic dystrophy.

Intracytoplasmic inclusion bodies of the thalamus in eight patients with myotonic dystrophy (MyD) were studied immunohistochemically. The intracytoplasmic inclusion bodies of the thalamus (thalamic inclusions, TIs) were strongly immunostained with anti-ubiquitin antibody (Ab) and some of them were mildly stained with anti-microtubule associated protein 1 (MAP 1) and anti-MAP 2 antibodies. However, TIs did not react with any of the following: anti-neurofilament protein Ab, anti-tau Ab, anti-paired helical filament Ab, anti-tubulin Abs (alpha and beta), anti-neuron-specific enolase Ab, anti-glial fibrillary acidic protein Ab, anti-synaptophysin Ab, anti-myelin basic protein Ab, anti-actin Ab and anti-phosphorylated epitope of neurofilaments Ab. Thus, our study demonstrates the unique immunohistochemistry of TIs in MyD which differentiates them from other intracytoplasmic inclusions in various neurodegenerative disorders.

Aged↗
PubMed1996

Synthesis and structure-activity relationships of 2-substituted D-tryptophan-containing peptidic endothelin receptor antagonists: importance of the C-2 substituent of the D-tryptophan residue for endothelin A and B receptor subtype selectivity.

Continuing studies on modifications of potent cyclic pentapeptide endothelin (ET) receptor antagonists, represented by BQ-123, and potent linear tripeptide derivative ET receptor antagonists, represented by BQ-788, are described herein. The introduction of D-tryptophan analogues with C-2 substituents in these peptidic ET antagonists resulted in potent ET receptor antagonists with various ETA/ETB subtype selectivity. Combined ETA/ETB receptor antagonists were found in both cyclic pentapeptide and linear tripeptide series with 2-halo- and 2-methyl-D-tryptophans. In contrast, compounds with 2-cyano-D-tryptophan were ETB receptor-selective antagonists. The C-2 substituent of the D-tryptophanyl residue appeared to be very important for the discrimination of ETA/ETB subtype selectivity of the antagonists. The potent ET receptor antagonists with various ETA/ETB subtype selectivity synthesized in this study may be useful tools for elucidating the physiological and pathophysiological roles of ET and ET receptors.

Animals↗
PubMed1996

Structural characterization and location of disulphide linkages of a potent vasodilatory peptide, recombinant maxadilan, by a multiple mass spectrometric approach.

A multiple mass spectrometric strategy using fast-atom bombardment (FAB) and matrix-assisted laser desorption/ionization (MALDI) has been used to confirm the sequence and to locate the disulfide linkages of recombinant maxadilan (r-maxadilan) (average molecular mass 7422.5 Da), a potent vasodilatory peptide from Lutzomyia longipalpis. MALDI measurements of intact r-maxadilan, its reduced form and its pyridylethylated form (p-maxadilan) indicated the presence of four Cys residues without major post-translational modifications. FAB and FAB-tandem mass spectrometry measurements of chymotryptic digests of p-maxadilan were sufficient to map the primary structure of p-maxadilan, though the complementary use of MALDI was necessary for complete mapping using Asp-N digestion due to a strong suppression observed in FAB. Assignment of the Cys-5-Cys-9 linkage was achieved by comparison of FAB mass spectra before and after reduction of tryptic digests of r-maxadilan. Since the molecular weight of the peptide fragment containing the Cys-18-Cys 55 linkage is more than 4000, MALDI measurement was indispensable for assignment of this linkage. The results fully support the value of the multiple mass spectrometric strategy in the structural characterization of peptides and proteins.

Amino Acid Sequence↗
PubMed1996

Identification of disulphide bonds in the refolding of bovine pancreatic RNase A.

BACKGROUND: Comprehension of the rules that govern the folding process is still far from satisfactory, though it is nevertheless clear that all the information required to define the folding is encoded in the amino acid sequence. In proteins that contain disulphide bonds, folding is associated with disulphide bond formation. Protein species with different numbers of disulphides tend to accumulate during the process; these species can be trapped in a stable form, by quenching any remaining free SH groups, and then characterized in order to identify the disulphide bonds formed. RESULTS: The refolding pathway of reduced and denatured RNase A has been studied using mass spectrometric strategies which allow identification of the formation and rearrangement of disulphide bonds during the process. When reoxidation was carried out in the presence of B M urea, producing the classic "scrambled' RNase, three native and 11 non-native disulphide bonds were identified. When the reoxidation was performed under nondenaturing conditions, the formation of several well defined non-native as well as native S-S bonds was observed at early stages of the refolding process. Under appropriate conditions, all four native disulphide bonds were identified at later stages of refolding and non-native disulphides were greatly diminished or non-existent. This stage corresponded with the almost complete recovery of biological activity of the protein. CONCLUSIONS: The results presented here show that both native and non-native disulphide bonds are formed during the refolding of reduced and denatured RNase A in vitro under different experimental conditions. Essentially 14 disulphide bonds were observed of the 2B theoretically possible cysteine couplings. Although this number constitutes a significant fraction of the theoretical total, the occurrence of only a subset of disulphides clearly indicates that the formation of the S-S bridges does not occur at random, even when reoxidation takes place under denaturing conditions.

Animals↗
PubMed1996

Neuronal loss in the medullary reticular formation in myotonic dystrophy: a clinicopathological study.

Respiratory insufficiency occurs frequently in patients with myotonic dystrophy (MyD). We have performed a quantitative study of neurons linked to respiratory function in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN) in seven patients with MyD and eight age-matched controls. Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The densities of neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in MyD without hypoventilation and controls. These data suggest the neuronal loss of the DCMN, VCMN, and SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.

Aged↗
PubMed1996

[Growth hormone prevents the steroid myopathy in rats].

To clarify whether growth hormone possesses the counteracting effect on steroid myopathy, we examined the influence of simultaneous administration of recombinant human growth hormone (rhGH) with glucocorticoid on the diameter of muscle fibers in rats. Female Sprague-Dawley rats weighing 120-140 g were divided into four groups. A group treated with glucocorticoid alone was subcutaneously injected with 5 mg/kg/day triamcinolone, a GH-treated group with 10 IU/kg/day rhGH alone, a steroid-GH-group with the same doses of glucocorticoid and rhGH. The control rats were injected with the vehicle alone. After 14 days of treatment, each rat was anesthetized with ether and subjected to muscle biopsy of extensor digitorum longus (EDL) and soleus. Administration of rhGH alone failed to affect the diameter of muscle fibers in either EDL or soleus. Glucocorticoid treatment caused a significant reduction in the diameter of muscle fibers in the EDL, compared with the control. In the type II muscle fibers of the EDL, simultaneous administration of rhGH attenuated glucocorticoid-induced muscle atrophy significantly. We concluded that GH administration would at least partially prevents steroid myopathy.

Animals↗
PubMed1995

Asparaginyl endopeptidase mapping of proteins with subsequent matrix-assisted laser desorption/ionization mass spectrometry.

A matrix-assisted laser desorption/ionization (MALDI) mass spectrometric mapping strategy for the identification and characterization of isolated and purified proteins is described. The method, which employs the combined usage of a new site-specific enzyme Asparaginyl endopeptidase (Asn-EP) for proteolysis, and MALDI for subsequent mass analysis, is capable of rapidly and sensitively examining the components of complex mixtures without any chromatographic or electrophoretic separation steps. Subpicomole sample quantities typically suffice to permit the confirmation of deduced primary structures and/or the identification of possible post-translational modifications. The data obtained should also prove useful for mass matching and sequence homology searching of computerised protein sequence data bases of known proteins.

Amino Acid Sequence↗
PubMed1995

Neuronal cell loss in the dorsal raphe nucleus and the superior central nucleus in myotonic dystrophy: a clinicopathological correlation.

A quantitative study of neurons in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) was performed in seven patients with myotonic dystrophy (MyD), five of whom showed hypersomnia, and in eight age-matched controls. The densities of neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and control subjects. There was an appreciable positive correlation in the density of neurons between the DRN and the SCN in all MyD patients. These data suggest that the neuronal loss of the DRN and the SCN is associated with the presence of hypersomnia in MyD.

Aged↗
PubMed1995

[A case of hereditary pressure-sensitive neuropathy, confirmed by a gene analysis].

A 21-year-old male patient with hereditary pressure-sensitive neuropathy (HPSN) is reported. He had been well and was working as a carpenter until February 6, 1993, when he developed difficulty in raising his left arm and numbness in the radial aspect of his left forearm in the morning. Left musculocutaneous nerve palsy, a left winged-scapula, absence of the left biceps and right Achilles reflexes, and distal dominant nerve conduction delay were positive findings. His father and younger brother showed similar conduction delays. Sural nerve biopsy revealed the presence of tomacula, segmental demyelination and thinly myelinated fibers. Gene analyses of the patient's cultured lymphoblastoid cells disclosed deletion of the marker 6G1 and peripheral myelin protein-22 (PMP-22) gene of a single allele of chromosome 17 in 90% of his intermitotic cells by in situ hybridization, and also a 57% gene dosage of PMP-22 of normal control using Southern blotting. These findings indicate the deletion in 17p11.2 of the genomic DNA of this patient, which is diagnostic for HPSN.

Adult↗