Heredodegenerative Disorders, Nervous System
Research News and Information

Sunday, November 22, 2009

Proteomic study of neuron and astrocyte cultures from senescence-accelerated mouse SAMP8 reveals degenerative changes.

5 Oct 2009 Senescence-accelerated prone (SAMP) strain 8 mice suffer an earlier development of cognitive age-related pathologies and a shorter life span than conventional mice. Protein alterations in astrocytes, in addition to those in neurons, may contribute ... Read more...

Ataxias with autosomal, X-chromosomal or maternal inheritance.

29 Jun 2009 Heredoataxias are a group of genetic disorders with a cerebellar syndrome as the leading clinical manifestation. The current classification distinguishes heredoataxias according to the trait of inheritance into autosomal dominant, autosomal ... Read more...

Combined hematopoietic and lentiviral gene-transfer therapies in newborn Twitcher mice reveal contemporaneous neurodegeneration and demyelination in Krabbe disease.

30 May 2009 This study characterized the therapeutic benefits of combining hematogenous cell replacement with lentiviral-mediated gene transfer of galactosylceramidase (GALC) in Twitcher mice, a bona fide model for Krabbe disease. Bone marrow cells and ... Read more...

Latest indexed articles for 'Heredodegenerative Disorders, Nervous System'

These are the very latest articles for this heading:

• Proteomic study of neuron and astrocyte cultures from senescence-accelerated mouse SAMP8 reveals degenerative changes. 5 Oct 2009
• Ataxias with autosomal, X-chromosomal or maternal inheritance. 29 Jun 2009
• Combined hematopoietic and lentiviral gene-transfer therapies in newborn Twitcher mice reveal contemporaneous neurodegeneration and demyelination in Krabbe disease. 30 May 2009
• Editorial. 'The muddle of embodiment'. 30 May 2009
• Late-onset HMSN 2: further evidence of genetic heterogeneity. 3 May 2009
• A 63-year-old woman with urinary incontinence and progressive gait disorder. 3 May 2009
• Megalencephalic leukoencephalopathy with subcortical cysts: a third confirmed case with literature review. 29 Apr 2009
• Early-onset neurodegenerative disease of the cerebellum and motor axons. 29 Apr 2009
• A 41-year-old woman with progressive leg weakness and numbness, dizziness, and myalgia. 5 Apr 2009
• Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease. 29 Mar 2009
• HSP70 interacting protein prevents the accumulation of inclusions in polyglutamine disease. 30 Jan 2009
• The neuronal expression of MYC causes a neurodegenerative phenotype in a novel transgenic mouse. 20 Jan 2009
• Iron accumulation in syndromes of neurodegeneration with brain iron accumulation 1 and 2: causative or consequential? 13 Jan 2009
• Dominant mutants of ceruloplasmin impair the copper loading machinery in aceruloplasminemia. 16 Dec 2008
• Testing for fragile X gene mutations throughout the life span. 24 Nov 2008
• Animal models of polyglutamine diseases and therapeutic approaches. 26 Oct 2008
• Mechanisms of RNA-mediated disease. 26 Oct 2008
• Unstable nucleotide repeat minireview series: a molecular biography of unstable repeat disorders. 26 Oct 2008
• Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: difficulties in the diagnosis. 23 Oct 2008
• Human receptor for activated protein kinase C1 associates with polyglutamine aggregates and modulates polyglutamine toxicity. 19 Oct 2008

See a longer list of these articles.

Technical information about 'Heredodegenerative Disorders, Nervous System'

Definition: Inherited disorders characterized by progressive atrophy and dysfunction of anatomically or physiologically related neurologic systems.

Descriptor UI: D020271

Alternative terms: Heredodegenerative Disorders, Nervous System; Degenerative Hereditary Diseases, Nervous System; Degenerative Hereditary Disorders, Nervous System; Hereditary Diseases, Neurodegenerative; Disease, Neurodegenerative Hereditary; Diseases, Neurodegenerative Hereditary; Hereditary Disease, Neurodegenerative; Neurodegenerative Hereditary Disease; Neurodegenerative Hereditary Diseases; Neurodegenerative Diseases, Hereditary; Disease, Hereditary Neurodegenerative; Diseases, Hereditary Neurodegenerative; Hereditary Neurodegenerative Disease; Neurodegenerative Disease, Hereditary; Hereditary-Degenerative Disorders, Nervous System; Hereditary Degenerative Disorders, Nervous System; Nervous System Degenerative Hereditary Diseases; Nervous System Diseases, Degenerative, Hereditary; Nervous System Hereditary Degenerative Diseases; Degenerative Disease, Nervous System, Hereditary; Hereditary Neurodegenerative Diseases;

Allowable Qualifiers: blood; cerebrospinal fluid; chemically induced; classification; complications; diagnosis; diet therapy; drug therapy; economics; embryology; enzymology; ethnology; etiology; genetics; history; immunology; metabolism; microbiology; mortality; nursing; epidemiology; parasitology; pathology; physiopathology; prevention & control; psychology; radiography; radionuclide imaging; radiotherapy; rehabilitation; surgery; therapy; ultrastructure; urine; veterinary; ultrasonography; virology;

Tree Number: C10.574.500; C16.320.400;

History Note: 2000