MCID: THR026
MIFTS: 19

the Organic Acidemias: an malady

Summaries for the Organic Acidemias: an

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19GeneReviews, 32MalaCards
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MalaCards: the Organic Acidemias: an is related to organic acidemia and metabolic acidosis. An important gene associated with the Organic Acidemias: an is GCDH (glutaryl-CoA dehydrogenase), and among its related pathways are Lysine degradation and tryptophan utilization II. The compounds 3-hydroxyglutaric acid and flavin-adenine dinucleotide have been mentioned in the context of this disorder.

GeneReviews summary for oa-overview

Aliases & Classifications for the Organic Acidemias: an

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19GeneReviews
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Aliases & Descriptions:

the organic acidemias: an 19


Related Diseases for the Organic Acidemias: an

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to the Organic Acidemias: an via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

idRelated DiseaseScoreTop Affiliating Genes
1organic acidemia30.2MCCC1, GCDH
2metabolic acidosis10.0HMGCL
3methylmalonic acidemia10.0HMGCL, MMACHC
4isovaleric acidemia10.0HMGCL, IVD
5homocystinuria10.0HMGCL, MMACHC
6beta-ketothiolase deficiency10.0HMGCL, ACAT1
7propionic acidemia10.0PCCA, PCCB
83 methylcrotonyl-coa carboxylase 1 deficiency10.0HMGCL, MCCC1, MCCC2
9metabolic syndrome x10.0ACAT1, PCCA, PCCB

Graphical network of diseases related to the Organic Acidemias: an:



Diseases related to the organic acidemias: an

Clinical Features for the Organic Acidemias: an

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Drugs & Therapeutics for the Organic Acidemias: an

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Sources:
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 the Organic Acidemias: an

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Anatomical Context for the Organic Acidemias: an

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Animal Models for the Organic Acidemias: an or affiliated genes

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Publications for the Organic Acidemias: an

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Genetic Variations for the Organic Acidemias: an

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Expression for genes affiliated with the Organic Acidemias: an

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with the Organic Acidemias: an

Search GEO for disease gene expression data for the Organic Acidemias: an.

Pathways for genes affiliated with the Organic Acidemias: an

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Sources:
29KEGG, 37NCBI BioSystems Database, 53Reactome
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Pathways related to the Organic Acidemias: an according to GeneCards/GeneDecks:

(show all 15)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8GCDH, ACAT1
2
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9.8ACAT1, GCDH
3
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9.8ACAT1, GCDH
4
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9.8ACAT1, GCDH
5
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9.8ACAT1, GCDH
6
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9.1PCCB, PCCA
7
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9.1PCCB, PCCA
88.8ACAT1, PCCA, PCCB
98.8PCCB, PCCA, ACAT1
10
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8.3ACAT1, HMGCL, PCCA, PCCB
118.2GCDH, IVD, MCCC2, MCCC1, ACAT1
12
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8.2ACAT1, HMGCL, MMACHC, MCCC1, MCCC2
13
Saturated fatty acid biosynthesis
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8.0PCCB, PCCA, MCCC2, MCCC1
14
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7.6PCCB, PCCA, MCCC2, MCCC1, MMACHC
15
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6.7ACAT1, HMGCL, MCCC1, MCCC2, PCCA, PCCB

Compounds for genes affiliated with the Organic Acidemias: an

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Sources:
44Novoseek, 11DrugBank, 24HMDB, 28IUPHAR
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Compounds related to the Organic Acidemias: an according to GeneCards/GeneDecks:

(show all 11)
idCompoundScoreTop Affiliating Genes
13-hydroxyglutaric acid44 1110.9GCDH, HMGCL
2flavin-adenine dinucleotide44 1110.6IVD, GCDH
3(E)-2-Methylglutaconic acid249.5MCCC2, MCCC1
43-methylglutaconyl-coa44 2410.3MCCC2, MCCC1
5pcca449.3PCCB, PCCA
6propionyl coa449.2PCCA, PCCB
73-methylcrotonyl-coa44 2410.2MCCC1, MCCC2, IVD
8acetyl-coa44 2410.0GCDH, IVD, HMGCL, ACAT1
9biotin44 11 2410.2PCCB, PCCA, MCCC2, MCCC1
10Adenosine triphosphate11 249.0MCCC1, MCCC2, PCCA, PCCB
11adp44 28 249.7PCCB, PCCA, MCCC2, MCCC1

GO Terms for genes affiliated with the Organic Acidemias: an

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16Gene Ontology
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Cellular components related to the Organic Acidemias: an according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1mitochondrial inner membraneGO:0057438.7ACAT1, HMGCL, MCCC1, GCDH
2mitochondrionGO:0057396.7GCDH, PCCB, MCCC2, MCCC1, MMACHC, HMGCL
3mitochondrial matrixGO:0057596.3ACAT1, GCDH, IVD, PCCB, PCCA, MCCC2

Biological processes related to the Organic Acidemias: an according to GeneCards/GeneDecks:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1cellular ketone body metabolic processGO:0469509.8HMGCL, ACAT1
2ketone body biosynthetic processGO:0469519.6ACAT1, HMGCL
3liver developmentGO:0018899.6ACAT1, HMGCL
4response to starvationGO:0425949.5HMGCL, ACAT1
5short-chain fatty acid catabolic processGO:0196269.2PCCB, PCCA
6fatty acid beta-oxidationGO:0066358.8PCCA, PCCB
7branched-chain amino acid catabolic processGO:0090838.8ACAT1, MCCC1, MCCC2, IVD
8leucine catabolic processGO:0065528.7HMGCL, MCCC1, MCCC2, IVD
9cellular lipid metabolic processGO:0442558.4ACAT1, HMGCL, PCCA, PCCB
10cellular nitrogen compound metabolic processGO:0346418.4GCDH, IVD, MCCC2, MCCC1, ACAT1
11biotin metabolic processGO:0067688.2PCCB, PCCA, MCCC2, MCCC1
12water-soluble vitamin metabolic processGO:0067677.9PCCB, PCCA, MCCC2, MCCC1, MMACHC
13vitamin metabolic processGO:0067667.8PCCB, PCCA, MCCC2, MCCC1, MMACHC
14small molecule metabolic processGO:0442816.3GCDH, IVD, PCCB, PCCA, MCCC2, MCCC1

Molecular functions related to the Organic Acidemias: an according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1flavin adenine dinucleotide bindingGO:0506609.7IVD, GCDH
2methylcrotonoyl-CoA carboxylase activityGO:0044859.4MCCC1, MCCC2
3fatty-acyl-CoA bindingGO:0000629.4HMGCL, GCDH
4biotin bindingGO:0093749.3MCCC1, PCCA
5biotin carboxylase activityGO:0040759.2PCCA, MCCC1
6propionyl-CoA carboxylase activityGO:0046589.2PCCA, PCCB

Products for genes affiliated with the Organic Acidemias: an

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for the Organic Acidemias: an

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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