Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism.

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Research Abstract Details 

Research Abstract Table of Contents

Jump to the:

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Abstract Text:

Shoichi Sasaki,Akiko Shirata,Kiyomi Yamane,Makoto Iwata,Shoichi Sasaki,Akiko Shirata,Kiyomi Yamane,Makoto Iwata,

We intensively examined the spinal cord of an autosomal recessive juvenile parkinsonism (ARJP) female patient with a homozygous exon 3 deletion in the parkin gene, anticipating a possible involvement of anterior horn neurons. Although the clinical features of the patient were consistent with parkinsonism as a result of parkin mutation, her tendon reflex was abolished in the lower limbs. This feature was in contrast with hyperreflexia, usually found in previous reports of ARJP. Histologically, on the level of the cervical, thoracic, and sacral spinal cord, anterior horn neurons were well preserved and normal. However, the lumbar spinal cord exhibited many swellings of proximal axons (spheroids) and degenerative changes in the somata of the large anterior horn neurons such as central chromatolysis, cystatin C-negative small eosinophilic inclusions, and eosinophilic Lewy body-like inclusions. Ultrastructurally, accumulations of neurofilaments and abnormal structures, such as inclusion bodies similar to skein-like inclusions and disorganized rough endoplasmic reticulum, were observed in the somata and neuronal processes. Lewy body-like inclusions in this study were positively immunostained for both alpha-synuclein and ubiquitin that closely resemble Lewy bodies, but are different from Lewy body-like inclusions negatively immunostained for alpha-synuclein in amyotrophic lateral sclerosis. These findings suggest that eosinophilic inclusions that closely resemble Lewy bodies may be formed in the spinal motor neurons of ARJP patients with parkin mutations and the motor neurons of these patients may be vulnerable to neurodegeneration.

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Publishing Authors By Initials

S Sasaki,A Shirata,K Yamane,M Iwata,S Sasaki,A Shirata,K Yamane,M Iwata,

For similar abstracts research abstracts see: abstracts research

PUBMED ID PMID:

MEDLINE DATE:

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuropathology : official journal of the Japanese

VOLUME: 28

Page Numbers: 74-80

Journal Abbreviation:

ISSN: 0919-6544

DAY: 20

MONTH: 11

YEAR: 2007

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 9606526

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Keywords Mesh Terms:

KEYWORDS:

MESH TERMS:

Chemical & Substance for Abstract: Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism. Information

Substance Name:

Registry Number:

Grant and Affiliation Information for Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism.

AFFILIATION: Department of Neurology, Neurological Institute, Tokyo Women's Medical University, Tokyo, Japan.

Country: Australia

AGENCY:

GRANT:

ACRONYM:

MEDLINETA: Neuropathology

REFSOURCE:

DATABASENAME:

ACCESSION NUMBER:

Number Hits: 0

Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism Related Publications

• A case of biopsy-proven leptomeningeal amyloidosis and intravenous Ig-responsive polyneuropathy associated with the Ala25Thr transthyretin gene mutation.
• A case of invasive micropapillary carcinoma of the breast.
• Bench to bedside strategies for optimizing neuroprotection following perinatal hypoxia-ischaemia in high and low resource settings.
• Complement activation on surfaces carrying amino groups.
• Complement activation on surfaces modified with ethylene glycol units.
• Differentiation of feline coronavirus type I and II infections by virus neutralization test.
• Fine structure and enzymatic degradation of poly[(R)-3-hydroxybutyrate] and stereocomplexed poly(lactide) nanofibers.
• Genome scan to detect genetic structure and adaptive genes of natural populations of Cryptomeria japonica.
• High titre of anti-Ascaris immunoglobulin E associated with bronchial asthma symptoms in 5-year-old rural Bangladeshi children.
• Induction of dopamine-releasing cells from primate embryonic stem cells enclosed in agarose microcapsules.
• Involvement of spinal motor neurons in parkin-positive autosomal recessive juvenile parkinsonism.
• Islet-encapsulation in ultra-thin layer-by-layer membranes of poly(vinyl alcohol) anchored to poly(ethylene glycol)-lipids in the cell membrane.
• Isolated limb sensory disturbance accompanied with sudden deafness from vertebral artery dissection: A case report.
• Lack of circadian regulation of melatonin rhythms in the sockeye salmon (Oncorhynchus nerka) in vivo and in vitro.
• Memory deficits in amyotrophic lateral sclerosis patients with dementia and degeneration of the perforant pathway A clinicopathological study.
• Microencapsulated feeder cells as a source of soluble factors for expansion of CD34(+) hematopoietic stem cells.
• Organization of pERK-immunoreactive cells in trigeminal spinal nucleus caudalis and upper cervical cord following capsaicin injection into oral and craniofacial regions in rats.
• Phosphorylation of ERK in trigeminal spinal nucleus neurons following passive jaw movement in rats with chronic temporomandibular joint inflammation.
• Purification of human plasma alpha 1-proteinase inhibitor and its inactivation by Pseudomonas aeruginosa elastase.
• Ribonucleic acid synthesis in nuclei isolated from rat liver.
• Scaffolds from electrospun polyhydroxyalkanoate copolymers: Fabrication, characterization, bioabsorption and tissue response.
• Structure and function of bacterial cytochrome c oxidase.
• The development of alternative vitrification solutions for microencapsulated islets.
• Turnover of proteoglycans by chick lens epithelial cells in cell culture and intact lens.
• Ultrastructural study on the specific binding of genetically engineered epidermal growth factor to type I collagen fibrils.
• [A simple screening test for risk of falls in the elderly]
• [Apraxia (author's transl)]
• [Objective markers for upper motor neuron involvement in amyotrophic lateral sclerosis]
• [Penicillin iontophoresis.]
• [Treatment of nasal allergies.]