7.2.4.3: (S)-methylmalonyl-CoA decarboxylase (sodium-transporting)
This is an abbreviated version!
For detailed information about (S)-methylmalonyl-CoA decarboxylase (sodium-transporting), go to the full flat file.
Word Map on EC 7.2.4.3
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7.2.4.3
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propionyl-coa
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veillonella
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parvula
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modestum
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propionigenium
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alcalescens
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biotin-containing
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extratemporal
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carboxyltransferase
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avidin-sepharose
- 7.2.4.3
- propionyl-coa
- veillonella
- parvula
- modestum
- propionigenium
- alcalescens
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biotin-containing
-
extratemporal
- carboxyltransferase
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avidin-sepharose
Reaction
Synonyms
Decarboxylase, methylmalonyl coenzyme A, EC 4.1.1.41, Methylmalonyl-CoA decarboxylase, Methylmalonyl-coenzyme A decarboxylase, MMCD, Propionyl coenzyme A carboxylase, Propionyl-CoA carboxylase, Sodium pump methylmalonyl-CoA decarboxylase
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General Information
General Information on EC 7.2.4.3 - (S)-methylmalonyl-CoA decarboxylase (sodium-transporting)
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physiological function
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14-year-old Asian-American male with propionic acidaemia shows propionyl-CoA carboxylase deficiency with mutations in PCCB: IVS7+2 T-G (c.763+2 T-G) and p.R410Q (c.1229 G-A), and residual enzyme activity which likely explains the patient's mild phenotype with isolated cardiomyopathy without any documented episodes of metabolic acidosis or evidence of any neurocognitive deficits
physiological function
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genes involved in precursor biosyntheses for avermectin or other antibiotics, such as methylmalonyl-CoA decarboxylase, are upregulated by 2.4fold in aveI mutant at day 10, which may potentially lead to increased supply of precursors into the antibiotics
physiological function
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propionic acidemia, a rare autosomal recessive disorder, is caused by deficiency in propionyl-CoA carboxylase, and is categorized into two forms: an early (neonatal) onset form, in which clinical symptoms are presented within the first 90 days of life, and a late onset form. Deficiency in propionyl-CoA carboxylase leads to accumulation of toxic substances in the body, that results in severe metabolic decompensation
physiological function
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the propionyl-CoA carboxylase pathway does not increase the production of FK506, a polyketide macrolide with immunosuppressant activity, in Streptomyces clavuligerus CKD1119
physiological function
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the propionyl-CoA carboxylase pathway does not increase the production of FK506, a polyketide macrolide with immunosuppressant activity, in Streptomyces clavuligerus CKD1119
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