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meglutol and Spastic Paraplegia, Hereditary

meglutol has been researched along with Spastic Paraplegia, Hereditary in 1 studies

Meglutol: An antilipemic agent which lowers cholesterol, triglycerides, serum beta-lipoproteins and phospholipids. It acts by interfering with the enzymatic steps involved in the conversion of acetate to hydroxymethylglutaryl coenzyme A as well as inhibiting the activity of HYDROXYMETHYLGLUTARYL COA REDUCTASES which is the rate limiting enzyme in the biosynthesis of cholesterol.
3-hydroxy-3-methylglutaric acid : A dicarboxylic acid that is glutaric acid in which one of the two hydrogens at position 3 is substituted by a hydroxy group, while the other is substituted by a methyl group. It has been found to accumulate in urine of patients suffering from HMG-CoA lyase (3-hydroxy-3-methylglutaryl-CoA lyase, EC 4.1.3.4) deficiency. It occurs as a plant metabolite in Crotalaria dura.

Spastic Paraplegia, Hereditary: A group of inherited diseases that share similar phenotypes but are genetically diverse. Different genetic loci for autosomal recessive, autosomal dominant, and x-linked forms of hereditary spastic paraplegia have been identified. Clinically, patients present with slowly progressive distal limb weakness and lower extremity spasticity. Peripheral sensory neurons may be affected in the later stages of the disease. (J Neurol Neurosurg Psychiatry 1998 Jan;64(1):61-6; Curr Opin Neurol 1997 Aug;10(4):313-8)

Research Excerpts

ExcerptRelevanceReference
"Costeff syndrome is a rare genetic neuro-ophthalmological syndrome consisting of early-onset bilateral optic atrophy along with a progressive complex motor disorder with elevated levels of urinary 3-methylglutaconic acid and 3-methylglutaric acid."3.81The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome. ( Anikster, Y; Ben-Zeev, B; Blumkin, L; Hassin-Baer, S; Lerman-Sagie, T; Lev, D; Schweiger, A; Sofer, S; Yahalom, G, 2015)

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
Sofer, S1
Schweiger, A1
Blumkin, L1
Yahalom, G1
Anikster, Y1
Lev, D1
Ben-Zeev, B1
Lerman-Sagie, T1
Hassin-Baer, S1

Other Studies

1 other study available for meglutol and Spastic Paraplegia, Hereditary

ArticleYear
The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome.
    American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics, 2015, Volume: 168B, Issue:3

    Topics: Adult; Chorea; Cognition Disorders; Executive Function; Female; Follow-Up Studies; Humans; Intellige

2015