dihydroxyphenylalanine has been researched along with Machado-Joseph Disease in 2 studies
Dihydroxyphenylalanine: A beta-hydroxylated derivative of phenylalanine. The D-form of dihydroxyphenylalanine has less physiologic activity than the L-form and is commonly used experimentally to determine whether the pharmacological effects of LEVODOPA are stereospecific.
dopa : A hydroxyphenylalanine carrying hydroxy substituents at positions 3 and 4 of the benzene ring.
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)
Excerpt | Relevance | Reference |
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"We performed [18F]6-fluoro-L-dopa (6-FD) and [11C]raclopride (RAC) PET studies in six patients with Machado-Joseph disease (MJD) (age, 17 to 61 years; duration of illness, 3 to 10 years), normal controls (n = 10 in 6-FD-PET, n = 8 in RAC-PET), and patients with idiopathic parkinsonism (n = 15 in 6-FD-PET)." | 7.69 | Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease. ( Calne, DB; Hashimoto, S; MacLeod, P; Rouleau, GA; Schulzer, M; Shinotoh, H; Silveira, I; Snow, BJ; Thiessen, B, 1997) |
"We performed [18F]6-fluoro-L-dopa (6-FD) and [11C]raclopride (RAC) PET studies in six patients with Machado-Joseph disease (MJD) (age, 17 to 61 years; duration of illness, 3 to 10 years), normal controls (n = 10 in 6-FD-PET, n = 8 in RAC-PET), and patients with idiopathic parkinsonism (n = 15 in 6-FD-PET)." | 3.69 | Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease. ( Calne, DB; Hashimoto, S; MacLeod, P; Rouleau, GA; Schulzer, M; Shinotoh, H; Silveira, I; Snow, BJ; Thiessen, B, 1997) |
"The subtype IV of Machado-Joseph disease (MJD), characterized by parkinsonism variably combined with ataxia, distal atrophy, and sensory loss, has been all but ignored in recent reports of MJD, including those describing the molecular biologic substrate of the disease." | 1.29 | Dopa-responsive parkinsonism phenotype of Machado-Joseph disease: confirmation of 14q CAG expansion. ( Lang, AE; Rogaeva, EA; St George-Hyslop, PH; Tuite, PJ, 1995) |
Timeframe | Studies, this research(%) | All Research% |
---|---|---|
pre-1990 | 0 (0.00) | 18.7374 |
1990's | 2 (100.00) | 18.2507 |
2000's | 0 (0.00) | 29.6817 |
2010's | 0 (0.00) | 24.3611 |
2020's | 0 (0.00) | 2.80 |
Authors | Studies |
---|---|
Tuite, PJ | 1 |
Rogaeva, EA | 1 |
St George-Hyslop, PH | 1 |
Lang, AE | 1 |
Shinotoh, H | 1 |
Thiessen, B | 1 |
Snow, BJ | 1 |
Hashimoto, S | 1 |
MacLeod, P | 1 |
Silveira, I | 1 |
Rouleau, GA | 1 |
Schulzer, M | 1 |
Calne, DB | 1 |
2 other studies available for dihydroxyphenylalanine and Machado-Joseph Disease
Article | Year |
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Dopa-responsive parkinsonism phenotype of Machado-Joseph disease: confirmation of 14q CAG expansion.
Topics: Chromosomes, Human, Pair 14; Dihydroxyphenylalanine; DNA; Female; Humans; Machado-Joseph Disease; Ma | 1995 |
Fluorodopa and raclopride PET analysis of patients with Machado-Joseph disease.
Topics: Adolescent; Adult; Caudate Nucleus; Cerebellum; Dihydroxyphenylalanine; Dopamine Antagonists; Female | 1997 |