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Literature summary for 1.2.4.4 extracted from

  • Skvorak, K.J.
    Animal models of maple syrup urine disease (2009), J. Inherit. Metab. Dis., 32, 229-246.
    View publication on PubMed

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
Mus musculus 5739
-

Organism

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
Mus musculus
-
heart
-
Mus musculus
-
kidney
-
Mus musculus
-
liver
-
Mus musculus
-
muscle
-
Mus musculus
-

Synonyms

Synonyms Comment Organism
BCKDH
-
Mus musculus
branched-chain keto acid dehydrogenase complex
-
Mus musculus

General Information

General Information Comment Organism
malfunction maple syrup urine disease is an autosomal recessive metabolic disorder resulting from a deficiency in the branched-chain alpha-keto acid dehydrogenase complex Mus musculus