6.4.1.3: propionyl-CoA carboxylase
This is an abbreviated version!
For detailed information about propionyl-CoA carboxylase, go to the full flat file.
Word Map on EC 6.4.1.3
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6.4.1.3
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propionic
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acidemia
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biotin-dependent
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carboxylases
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methylmalonyl-coa
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acidosis
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methylmalonic
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3-hydroxypropionate
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methylcitrate
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propionylation
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hyperammonemia
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holocarboxylase
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3-methylcrotonyl-coa
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acidurias
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biotin-containing
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carboxyltransferase
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biotin-deficient
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hyperglycinemia
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beta-methylcrotonyl-coa
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biotin-binding
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odd-chain
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anaplerosis
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transcarboxylase
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3-hydroxyisovaleric
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propionylcarnitine
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synthesis
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analysis
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medicine
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apocarboxylases
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biotinidase
- 6.4.1.3
- propionic
- acidemia
-
biotin-dependent
- carboxylases
- methylmalonyl-coa
- acidosis
-
methylmalonic
- 3-hydroxypropionate
-
methylcitrate
-
propionylation
-
hyperammonemia
- holocarboxylase
- 3-methylcrotonyl-coa
- acidurias
-
biotin-containing
- carboxyltransferase
-
biotin-deficient
- hyperglycinemia
- beta-methylcrotonyl-coa
-
biotin-binding
-
odd-chain
-
anaplerosis
- transcarboxylase
-
3-hydroxyisovaleric
- propionylcarnitine
- synthesis
- analysis
- medicine
- apocarboxylases
- biotinidase
Reaction
Synonyms
AccA3-PccB complex, acetyl-CoA/propionyl-CoA carboxylase, Carboxylase, propional coenzyme A (adenosine triphosphate-hydrolyzing), LA_2736-LA_2735, Pcase, PCC, PccA, PccA-1, PCCase, PccB, PccB-1, pccBC, PccE, Propanoyl-CoA:carbon dioxide ligase, Propionyl coenzyme A carboxylase, Propionyl coenzyme A carboxylase (adenosine triphosphate-hydrolyzing), Propionyl coenzyme A carboxylase (ATP-hydrolyzing), Propionyl-CoA carboxylase, propionyl-coenzyme A carboxylase
ECTree
6.4.1.3 propionyl-CoA carboxylaseAdvanced search results
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results (Application
Application on EC 6.4.1.3 - propionyl-CoA carboxylase
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APPLICATION 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
analysis
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method to analyze propionyl-CoA carboxylase activity in phytohemagglutinin stimulated lymphocytes using high performance liquid chromatography. Propionyl-CoA carboxylase activity is unaffected even when lymphocytes are isolated and phytohemagglutinin stimulated after a whole blood sample has been stored at 4°C for 5 days, and the method is useful for the confirmation of propionic acidemia in individuals, and prenatal diagnosis and genetic counseling for the affected families
medicine
synthesis
expression of the propionyl-CoA carboxylase complex from Streptomyces coelicolor supports the highest levels of heterologous polyketide production in Escherichia coli. The molar yield of 6-deoxyerythronolide B of Escherichia coli, harboring the wild-type Streptomyces coelicolor propionyl-CoA carboxylase, is 1.2%
medicine
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stringent model of propionyl CoA carboxylase subunit A deficiency, where homozygous knock-out mice are born, but die within 36 hours. Injection of vectors expressing propionyl CoA carboxylase subunit A significantly increases the lifespan for both unmodified and polyethylene glycol modified vectors, but the rescue is transient
medicine
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living-donor liver transplantation is an effective treatment modality in patients with congenial metabolic liver disease, i. e., propionic acidemia caused by deficiency in propionyl-CoA carboxylase
medicine
treatment of propionic acidemia by import of a biologically active, fully assembled propionyl-CoA carboxylase dodecamer, posttranslationally chemically conjugated with the HIV TAT peptide. TAT-propionyl-CoA carboxylase is transferred into isolated mitochondria, as well as into mitochondria of patient fibroblasts . A single-dose intraperitoneal injection into propionyl-CoA carboxylase-deficient mice decreases the propionylcarnitine/acetylcarnitine (C3/C2) ratio toward the normal level
