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Pathways
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Ontology Browser
Term:
3-methylcrotonyl CoA carboxylase 1 deficiency pathway (PW:0002576)
Annotations: Rat: (30) Mouse: (30) Human: (30) Chinchilla: (30) Bonobo: (30) Dog: (30) Squirrel: (30) Pig: (30) Naked Mole-rat: (28) Green Monkey: (29)
Parent Terms Term With Siblings Child Terms
3-methylcrotonyl CoA carboxylase 1 deficiency pathway  
An inheritied condition resulting from alterations in the degradation of branched-chain amino acid due to defects in the alpha subunit of methylcrotonyl CoA carboxylase (MCCC1). MCCC is a biotin-requiring enzyme in the mitochondria.
3-methylcrotonyl CoA carboxylase 2 deficiency pathway 
argininosuccinic aciduria pathway  
carbamoyl phosphate synthetase I deficiency pathway  
citrullinemia pathway  
hyperargininemia pathway  
ornithine translocase deficiency pathway  

Synonyms
Related Synonyms: 3-methylcrotonyl CoA carboxylase type I deficiency pathway ;   SMP:00237
Definition Sources: https://en.wikipedia.org/wiki/Methylcrotonyl-CoA_carboxylase "wikipedia" "wikipedia", OMIM:210200

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