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Biomedical subjects

S Kusunoki

Publications and source records attributed to S Kusunoki.

At least 55 records · Page 3Linked to original sources
PubMed1999

Anti-GQ1b IgG antibody is associated with ataxia as well as ophthalmoplegia.

Close association between the increase in anti-GQ1b immunoglobulin G (IgG) antibody and ophthalmoplegia in Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) has been reported. We investigated whether anti-GQ1b IgG antibody also is associated with ataxia, another of the MFS triad. Of 149 patients who had anti-GQ1b IgG antibody without profound weakness, 144 showed ophthalmoplegia (120 showed both ophthalmoplegia and ataxia; 24, ophthalmoplegia without ataxia). In contrast, five showed ataxia without ophthalmoplegia. Some large neurons of the dorsal root ganglia were immunostained with anti-GQ1b monoclonal antibody. Anti-GQ1b IgG antibody may thus be associated with ataxia as well as ophthalmoplegia. Ataxia may be due to its binding to a subset of primary sensory neurons.

Antibodies↗
PubMed1999

Clinicopathological study of an autopsy case with sensory-dominant polyradiculoneuropathy with antiganglioside antibodies.

A previously reported patient presenting sensory-dominant neuropathy with antiganglioside antibodies, bound preferentially to polysialogangliosides including GD1b, was autopsied. While axonal degeneration was predominant in the sural nerve, many demyelinated fibers were present in the spinal roots. Dorsal roots had undergone significant damage. These pathological findings were well correlated with the electrophysiological results showing decreased F-wave conduction velocities and conduction blocks in motor nerves and decreased or absent sensory action potentials in sensory nerves, with distribution of GD1b in nerve tissues such as dorsal root ganglia and paranodal myelin in the ventral and dorsal roots.

Aged↗
PubMed1999

Sensorimotor polyneuropathy associated with chronic lymphocytic leukemia, IgM antigangliosides antibody and human T-cell leukemia virus I infection.

A 65-year-old man presented with a sensorimotor polyneuropathy associated with B-cell chronic lymphocytic leukemia (CLL) and immunoglobulin M (IgM) antibody to various gangliosides. Electrophysiological studies denoted significant abnormalities of motor and sensory nerve conduction. Although the pathology of sural nerve biopsy looked minimally affected, immunohistochemical studies showed specific binding of IgM to the human peripheral nerve. Our patient also had high titer of antibody to human T-cell leukemia virus I (HTLV-I) in both serum and cerebrospinal fluid (CSF), which might activate B-cell-mediated immunity and facilitate the production of IgM antibody. The other unique feature is the reactivity of antibody to gangliosides. The patient had IgM antibody reactivities to gangliosides with disialosyl residue such as GT1b, GQ1b and GD3, but not to GD1b. IgM antibody to gangliosides with disialosyl residue has been reported in ataxic symptoms, but our patient failed to demonstrate ataxia. Without reactivity to GD1b, sensory ataxic neuropathy might not develop even in the presence of antibody reactive to other gangliosides with disialosyl residue.

Aged↗
PubMed1999

Hbox1 and Hbox7 are involved in pattern formation in sea urchin embryos.

In spite of their potential importance in evolution, there is little information about Hox genes in animal groups that are related to ancestors of deuterostome. It has been reported that only two Hox genes (Hbox1 and Hbox7) are expressed significantly in sea urchin embryos. Expression of Hbox1 protein is restricted to the aboral ectoderm, and Hbox7 expression is restricted to oral ectoderm, endoderm and secondary mesenchyme cells in sea urchin embryos after the gastrula stage. With the aim of gaining insight into the role of Hbox1 and Hbox7 in sea urchin development, Hbox1 and Hbox7 overexpression experiments were performed. Overexpression of Hbox1 repressed the development of oral ectoderm, endoderm and mesenchyme cells. On the contrary, overexpression of Hbox7 repressed the development of aboral ectoderm and primary mesenchyme cells. The data suggest that Hbox1 and Hbox7 are expressed in distinct non-overlapping territories, and overexpression of either one inhibits territory-specific gene expression in the domain of the other. It is proposed that an important function of both Hbox1 and Hbox7 genes is to maintain specific territorial gene expression by each one, in its domain of expression, while repressing the expression of the other in this same domain.

Amino Acid Sequence↗
PubMed1999

Monospecific anti-GD1b IgG is required to induce rabbit ataxic neuropathy.

Of 22 rabbits sensitized with GD1b, 12 developed experimental sensory ataxic neuropathy. The affected rabbits had a higher level of serum IgG monospecific to GD1b than the unaffected ones. The GD1b-positive neuronal cytoplasms of rabbit dorsal root ganglia had larger diameters than the negative ones. IgG antibody monospecific to GD1b may preferentially bind to large primary sensory neurons, causing sensory ataxic neuropathy.

Animals↗
PubMed1999

[Antiganglioside antibodies in the pathogenesis of autoimmune neuropathies].

Antiganglioside antibodies are frequently detected in sera from patients with autoimmune neuropathies, such as Guillain-Barré syndrome, Miller Fisher syndrome, IgM paraproteinemic neuropathy, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. In the acute phase sera from GBS patients, antiganglioside antibodies are detected in 60-70%. Ganglioside antigens recognized by serum antibodies are varied from case to case. IgG antibody against GQ1b ganglioside is specifically raised in sera from patients with Miller Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia. That antibody may bind to the paranodal myelin of oculomotor, trochlear and abducens nerves, where GQ1b ganglioside is specifically localized, to cause ophthalmoplegia. IgM M-protein which recognizes the disialosyl residue of GD1b is specifically associated with sensory ataxic neuropathy. The IgM M-protein may bind to the primary sensory neurons, where GD1b ganglioside is localized, to cause sensory disturbance. After we confirmed the localization of GD1b in the rabbit primary sensory neurons, we sensitized rabbits with GD1b and induced sensory ataxic neuropathy in them. This is the first established animal model of autoimmune neuropathy induced by sensitization with ganglioside. Some antiganglioside antibodies may determine the clinical phenotype of neuropathy by binding specifically to the ganglioside antigens which have unique localization.

Animals↗
PubMed1999

[Guillain-Barré syndrome with cerebellar symptoms and elevated serum anti-GD1b IgG antibody].

We reported a 46-year-old woman with Guillain-Barré syndrome (GBS) after suffering from common cold. She also had cerebellar symptoms; ataxic speech, poor finger-nose and heel-knee tests, dysmetria, dysdiadochokinesis, poor one foot standing, positive Mann's test, ataxic gait, and poor tandem gait without Romberg's sign, and sensory disturbance. We ruled out other diseases with cerebellar symptoms; for example, Wernicke encephalopathy, multiple sclerosis, cerebellar vascular disease and encephalitis in the brain stem and cerebellum. Anti-GD1bIgG antibody was elevated in her serum in the acute phase. She was treated with immuno-adsorption therapy (TR-350) in the acute phase. The antibody titer decreased with clinical improvement after immuno-adsorption therapy. The involvement of the anti-GD1bIgG antibody in the pathogenetic mechanism of peripheral neuropathy and cerebellar symptoms was suggested.

Autoantibodies↗
PubMed1999

[A case of pharyngeal-cervical-brachial variant of Guillain-Barré syndrome with positive anti-galactocerebroside (Gal-C) IgM antibody].

A 49-year-old man presented with hoarseness, dysphagia, muscle atrophy and weakness of deltoid, trapezius, sternocleidomastoid, rhomboid, anterior serratus, infraspinatus and supraspinatus. Anti-Gal-C IgM antibody was positive in the serum. The other antiganglioside antibodies (GM1, GM2, GM3, GD1a, GD1b, GD3, GT1a, GT1b, GQ1b, GA1, GalNAc-GD1a, GM1b) were negative. Patient contracted pneumonia but whether it was due to mycoplasma was not evident. Plasmapheresis improved his clinical state including a decrease of the antibody. This case was diagnosed pharyngeal-cervical-brachial variant of Guillain-Barré syndrome, and anti-Gal-C antibody seemed to be correlated with the pathogenesis of this syndrome. Gal-C is a major glycolipid of myelin and the cell membrane of the myelin-forming cell (oligodendrocytes and Schwann cells) and is free of specific localization and distribution. The mechanism how the anti-Gal-C IgM antibody induced bulbar paralysis and the symptoms localizing neck and upper limbs remains to be known.

Autoantibodies↗
PubMed1999

[Pathogenetic mechanism of experimental autoimmune neuropathy induced by sensitization with a ganglioside].

Antiganglioside antibodies are frequently present in sera from patients with autoimmune neuropathies. To elucidate the pathogenetic mechanisms of autoimmune neuropathies mediated by antiganglioside antibodies, we established a rabbit model of sensory ataxic neuropathy induced by sensitization with ganglioside GD1b (GD1b-SAN). Degeneration of primary sensory neurons extending the central axons to the dorsal column of spinal cord was observed pathologically. No lymphocytic cell infiltration was seen. Anti-GD1b antibody therefore should be an essential factor to induce GD1b-SAN. In sera from rabbits immunized with GD1b, two types of antibodies were present; antibodies monospecific to GD 1 b and those cross-reactive with GM1. Of 22 rabbits sensitized with GD1b, 12 developed GD1b-SAN. The level of IgG antibody monospecific to GD1b was higher in the sera from affected rabbits than in those from unaffected ones. The GD1b-positive neuronal cytoplasms of rabbit dorsal root ganglia had larger diameters than the GD1b-negative ones. Markedly reduced expression of trkC in dorsal root ganglia from rabbits with GD1b-SAN in acute phase was found. IgG antibody monospecific to GD1b may cause GD1b-SAN by preferentially binding to large primary sensory neurons mediating proprioceptive sensation. Anti-GD1b antibody-mediated downregulation of trkC expression could be one of the pathogenesis of GD 1 b-SAN.

Animals↗
PubMed1998

The pattern of antiganglioside antibody reactivities producing myelinated nerve conduction block in vitro.

We studied the pattern of human antiganglioside antibody reactivities causing an acute conduction block in rat myelinated nerve fibers, using an in vitro preparation of the sciatic-tibial nerve. With the aid of complements, IgM antibodies reacting with the terminal disaccharide of galactose (beta1-3)N-acetylgalactosamine produced the block. These findings may help us to understand the mechanism in which the conduction block occurs in neuropathies associated with antiganglioside antibodies.

Action Potentials↗
PubMed1998

Severe motor-dominant neuropathy with IgM M-protein binding to the NeuAcalpha2-3Galbeta- moiety.

We report the occurrence of a relapsing, severe predominantly motor neuropathy in a 75-year-old man with an IGM-K M-protein binding to gangliosides GM2, GM3, GM4, GD1a, GT1b and LM1. Motor nerve conduction velocities were slowed with conduction block. A superficial peroneal nerve biopsy specimen revealed segmental demyelination and remyelination. The patient improved after repeated plasma exchanges, and the antibody titer decreased in association with clinical recovery. This IgM M-protein has a unique, previously unreported binding specificity for terminal NeuAcalpha2-3Galbeta- moiety in common to all gangliosides bound by the antibody except GM2. M-proteins with this affinity may be involved in the pathogenesis of this and other cases of motor-dominant demyelinating neuropathy.

Aged↗
PubMed1998

Dorsal root ganglia-specific expression of the beta-galactoside alpha1,2-fucosyltransferase genes in rabbits.

The neurons of dorsal root ganglia (DRG) mediate several sensation modalities. The carbohydrate antigens on DRG neurons differ with the sensation modalities that subsets of neurons convey. Despite the important roles of gangliosides and glycoproteins in neuronal differentiation and neuritogenesis of the mammalian nervous system, little is known about the mechanisms underlying the regulation of glycosylation. We previously demonstrated the expression of H-blood type antigens (Fuc alpha1, 2Gal beta) on rabbit DRG neurons of small diameter and dramatic changes in H antigens during the perinatal period. To investigate the possible biological roles and regulatory mechanisms of H antigens, we recently cloned three types of rabbit alpha1,2-fucosyltransferase gene that catalyze the biosynthesis of H antigens. Here, we analyze the expression of these genes, RFT-I, II, and III, in rabbit DRG. The H-type alpha1,2-fucosyltransferase gene, RFT-I, was expressed in DRG in late embryos to adult rabbits, as detected on northern blotting. The other two secretor-type alpha1,2-fucosyltransferase genes, RFT-II and III, were observed to be expressed in late embryonic DRG on RT-PCR analysis but were not detectable on northern blotting. The expression of the H-type alpha1,2-fucosyltransferase gene was analyzed by in situ hybridization and was found to be abundant in small-diameter DRG neurons. These results indicate that the H-type alpha1,2-fucosyltransferase gene plays a major role in the regulation of the H antigen expression in DRG during the perinatal period.

Animals↗
PubMed1998

Anti-GQ1b ganglioside antibody and ophthalmoplegia of undetermined cause.

BACKGROUND/AIM: Serum antibody against ganglioside GQ1b is reported to be closely associated with immune mediated ophthalmoplegia in the Fisher and Guillain-Barré syndromes. Its presence against glycolipids, in particular ganglioside GQ1b, was investigated in patients with ophthalmoplegia of unknown origin. METHODS: 16 patients with ophthalmoplegia, the cause of which could not be confirmed from clinical findings or diagnostic testing, were tested. 34 patients who had ophthalmoplegia of definite cause, 16 healthy people, and 23 patients with typical Fisher syndrome served as the controls. The ELISA was used to check for serum antibodies against glycolipids in all study participants. RESULTS: Two of the 16 patients with ophthalmoplegia of unknown cause had serum IgG antibody against GQ1b but not against other glycolipids, and 22 of the 23 patients with typical Fisher syndrome had this antibody. No anti-GQ1b antibodies were found in the patients with ophthalmoplegia of definite cause or in the normal controls. CONCLUSION: A common underlying cause appears to bring about the pathogenesis of palsy in Fisher syndrome and in the ophthalmoplegia with positive anti-GQ1b IgG antibody, called atypical Fisher syndrome. This antibody may prove a useful clinical marker for differentiating Fisher syndrome, typical and atypical, in patients with ophthalmoplegia.

Adult↗