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3-nitropropionic acid and Machado-Joseph Disease

3-nitropropionic acid has been researched along with Machado-Joseph Disease in 1 studies

3-nitropropionic acid: succinate dehydrogenase inactivator; biosynthesized by FABACEAE plants from ASPARAGINE
3-nitropropanoic acid : A C-nitro compound that is propanoic acid in which one of the methyl hydrogens has been replaced by a nitro group.

Machado-Joseph Disease: A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96)

Research Excerpts

ExcerptRelevanceReference
"Machado-Joseph disease (MJD), also known as Spinocerebellar Ataxia type 3, is an inherited dominant autosomal neurodegenerative disorder."1.38Compromised mitochondrial complex II in models of Machado-Joseph disease. ( Laço, MN; Oliveira, CR; Paulson, HL; Rego, AC, 2012)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's0 (0.00)29.6817
2010's1 (100.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Laço, MN1
Oliveira, CR1
Paulson, HL1
Rego, AC1

Other Studies

1 other study available for 3-nitropropionic acid and Machado-Joseph Disease

ArticleYear
Compromised mitochondrial complex II in models of Machado-Joseph disease.
    Biochimica et biophysica acta, 2012, Volume: 1822, Issue:2

    Topics: Animals; Ataxin-3; Cell Death; Cell Line; Cell Line, Transformed; Cells, Cultured; Cerebellum; Disea

2012