3MGA
MCID: 3MT007
MIFTS: 22

3-Methylglutaconic Aciduria (3MGA) malady

Summaries for 3-Methylglutaconic Aciduria

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21Genetics Home Reference, 63Wikipedia, 32MalaCards
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Genetics Home Reference:21 The name 3-methylglutaconic aciduria is used to describe five different disorders that impair the functioning of energy-producing centers within cells (mitochondria). As a result of this impairment, pathways that aid in mitochondrial function are disrupted and large amounts of particular acids (3-methylglutaconic acid and 3-methylglutaric acid) build up and are excreted in the urine.

MalaCards: 3-Methylglutaconic Aciduria, also known as 3-@methylglutaconic aciduria, type i, is related to opa3-related 3-methylglutaconic aciduria and barth syndrome. An important gene associated with 3-Methylglutaconic Aciduria is OPA3 (optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)), and among its related pathways are Mitochondrial Protein Import and Branched-chain amino acid catabolism. The compounds ryanodine and 3-methylglutaconic acid have been mentioned in the context of this disorder.

Wikipedia:63 3-Methylglutaconic aciduria (MGA) is used to describe at least five disorders that impair the body\'s... more...

Aliases & Classifications for 3-Methylglutaconic Aciduria

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21Genetics Home Reference, 60UMLS
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Aliases & Descriptions:

3-methylglutaconic aciduria 21
3-@methylglutaconic aciduria, type i 60
3mga 21


Related Diseases for 3-Methylglutaconic Aciduria

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to 3-Methylglutaconic Aciduria:



Diseases related to 3-methylglutaconic aciduria

Clinical Features for 3-Methylglutaconic Aciduria

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Drugs & Therapeutics for 3-Methylglutaconic Aciduria

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for 3-Methylglutaconic Aciduria

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Anatomical Context for 3-Methylglutaconic Aciduria

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Animal Models for 3-Methylglutaconic Aciduria or affiliated genes

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Publications for 3-Methylglutaconic Aciduria

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Genetic Variations for 3-Methylglutaconic Aciduria

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Expression for genes affiliated with 3-Methylglutaconic Aciduria

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with 3-Methylglutaconic Aciduria

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Pathways for genes affiliated with 3-Methylglutaconic Aciduria

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53Reactome, 37NCBI BioSystems Database, 29KEGG
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Pathways related to 3-Methylglutaconic Aciduria according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8TAZ, DNAJC19
2
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9.2MCCC2, EHHADH, AUH

Compounds for genes affiliated with 3-Methylglutaconic Aciduria

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44Novoseek, 28IUPHAR, 59Tocris Bioscience, 24HMDB
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Compounds related to 3-Methylglutaconic Aciduria according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ryanodine44 28 5911.6RYR1, DMPK
23-methylglutaconic acid449.5OPA3, AUH
33-methylglutaconyl-coa44 2410.2MCCC2, EHHADH, AUH
43-methylcrotonyl-coa44 2410.1AUH, EHHADH, MCCC2

GO Terms for genes affiliated with 3-Methylglutaconic Aciduria

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16Gene Ontology
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Cellular components related to 3-Methylglutaconic Aciduria according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1sarcoplasmic reticulum membraneGO:0330179.3RYR1, DMPK
2mitochondrial inner membraneGO:0057439.1TMEM70, TAZ, DNAJC19
3mitochondrionGO:0057397.7SERAC1, AUH, OPA3, EHHADH, DNAJC19, MCCC2

Biological processes related to 3-Methylglutaconic Aciduria according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1leucine catabolic processGO:0065529.6MCCC2, AUH
2muscle contractionGO:0069369.5TAZ, RYR1
3branched-chain amino acid catabolic processGO:0090839.4MCCC2, AUH

Molecular functions related to 3-Methylglutaconic Aciduria according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1enoyl-CoA hydratase activityGO:0043009.6EHHADH, AUH

Products for genes affiliated with 3-Methylglutaconic Aciduria

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Sources for 3-Methylglutaconic Aciduria

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet