Page last updated: 2024-10-21

meglutol and Optic Atrophy

meglutol has been researched along with Optic Atrophy in 3 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.

Optic Atrophy: Atrophy of the optic disk which may be congenital or acquired. This condition indicates a deficiency in the number of nerve fibers which arise in the RETINA and converge to form the OPTIC DISK; OPTIC NERVE; OPTIC CHIASM; and optic tracts. GLAUCOMA; ISCHEMIA; inflammation, a chronic elevation of intracranial pressure, toxins, optic nerve compression, and inherited conditions (see OPTIC ATROPHIES, HEREDITARY) are relatively common causes of this condition.

Research Excerpts

ExcerptRelevanceReference
"Eleven new patients of Iraqi-Jewish origin with bilateral optic atrophy, neurological abnormalities ('optic atrophy plus' syndrome) and 3-methylglutaconic aciduria (type III) are described."7.693-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome. ( Costeff, H; Elpeleg, ON; Gibson, KM; Joseph, A; Shental, Y; Weitz, R, 1994)
"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)
"Eleven new patients of Iraqi-Jewish origin with bilateral optic atrophy, neurological abnormalities ('optic atrophy plus' syndrome) and 3-methylglutaconic aciduria (type III) are described."3.693-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome. ( Costeff, H; Elpeleg, ON; Gibson, KM; Joseph, A; Shental, Y; Weitz, R, 1994)

Research

Studies (3)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's2 (66.67)18.2507
2000's0 (0.00)29.6817
2010's1 (33.33)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, T2
Hassin-Baer, S1
Elpeleg, ON1
Costeff, H1
Joseph, A1
Shental, Y1
Weitz, R1
Gibson, KM1

Other Studies

3 other studies available for meglutol and Optic Atrophy

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
3-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome.
    Developmental medicine and child neurology, 1994, Volume: 36, Issue:2

    Topics: Adolescent; Adult; Child; Child, Preschool; Cognition Disorders; Female; Glutarates; Humans; Iraq; J

1994
Behr syndrome.
    Pediatric neurology, 1995, Volume: 12, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child; Glutarates; Humans; Meglutol

1995