Methylcrotonoyl-CoA Carboxylase 2 (Beta) Protéines (MCCC2)

MCCC2 encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. De plus, nous expédions Methylcrotonoyl-CoA Carboxylase 2 (Beta) Anticorps (73) et Methylcrotonoyl-CoA Carboxylase 2 (Beta) Kits (1) et beaucoup plus de produits pour cette protéine.

afficher tous les protéines Gène GeneID UniProt
MCCC2 64087 Q9HCC0
Rat MCCC2 MCCC2 361884 Q5XIT9
MCCC2 78038 Q3ULD5
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Top Methylcrotonoyl-CoA Carboxylase 2 (Beta) Protéines sur anticorps-enligne.fr

Showing 6 out of 7 products:

Catalogue No. Origin Source Conjugué Images Quantité Livraison Prix Détails
Cellules d'insectes Humain His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 60 Days
$9,626.73
Détails
Cellules d'insectes Souris His tag „Crystallography Grade“ protein due to multi-step, protein-specific purification process 1 mg 60 Days
$9,626.73
Détails
Wheat germ Humain GST tag 10 μg 11 to 12 Days
$414.29
Détails
HEK-293 Cells Humain Myc-DYKDDDDK Tag Validation with Western Blot 20 μg 11 Days
$888.80
Détails
Escherichia coli (E. coli) Humain Inconjugué 100 μg 15 to 18 Days
$762.00
Détails
Escherichia coli (E. coli) Souris Inconjugué 100 μg 15 to 18 Days
$794.00
Détails

MCCC2 Protéines protéines par origine et source

Origin Exprimée danse Conjugué
Human , , ,
, ,
Mouse (Murine) ,

Plus protéines pour Methylcrotonoyl-CoA Carboxylase 2 (Beta) (MCCC2) partenaires d'interaction

Human Methylcrotonoyl-CoA Carboxylase 2 (Beta) (MCCC2) interaction partners

  1. This study reports data mainly obtained from the Portuguese newborn screening program collected over a ten-year period. Analysis of the MCCC1 and MCCC2 genes yielded 26 previously unreported mutations and a variant of clinically unknown significance.

  2. Novel mutation in MCCC2 gene was identified in Chinese population.

  3. Mutation in 3-methylcrotonyl CoA carboxylase 2 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency.

  4. study reports eight different mutant alleles of MCCC1 or MCCC2 including six novel mutations in Korean patients with 3-methylcrotonyl-CoA carboxylase (MCC) deficiency

  5. identified two novel MCCA and four novel MCCB mutant alleles from five MCC-deficient patients

  6. factors other than the genotype at the MCCA and MCCB loci have a major influence on the phenotype of MCC deficiency

  7. The amino-termini containing 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis.

  8. The Kd value of soraphen A for the BC domains of human ACC1 and ACC2 is 1 nM. This high binding affinity is mainly due to the extensive interactions between soraphen A and the human biotin carboxylase domain

  9. Molecular analyses revealed novel mutations in one of the causative genes, MCCA or MCCB, in all five of the MCC deficiency patients

Arabidopsis thaliana Methylcrotonoyl-CoA Carboxylase 2 (Beta) (MCCC2) interaction partners

  1. Studies indicate that mutations in either 3-methylcrotonyl CoA carboxylase MCCA (At1g03090) or MCCB (At4g34030) block mitochondrial Leu catabolism, as inferred from the increased accumulation of Leu.

Profil protéine Methylcrotonoyl-CoA Carboxylase 2 (Beta) (MCCC2)

Profil protéine

This gene encodes the small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.

Gene names and symbols associated with MCCC2

  • methylcrotonoyl-CoA carboxylase 2 (beta) (mccc2)
  • methylcrotonoyl-CoA carboxylase 2 (MCCC2)
  • methylcrotonoyl-CoA carboxylase 2 (mccc2)
  • methylcrotonoyl-CoA carboxylase 2 S homeolog (mccc2.S)
  • methylcrotonoyl-CoA carboxylase 2 (Mccc2)
  • 3-methylcrotonyl-CoA carboxylase (MCCB)
  • methylcrotonoyl-Coenzyme A carboxylase 2 (beta) (Mccc2)
  • 3-methylcrotonyl-CoA carboxylase Protéine
  • 4930552N12Rik Protéine
  • F28A23.210 Protéine
  • F28A23_210 Protéine
  • MCCB Protéine
  • si:dkey-57m14.1 Protéine
  • zgc:85685 Protéine

Protein level used designations for MCCC2

methylcrotonoyl-CoA carboxylase beta chain, mitochondrial , methylcrotonoyl-Coenzyme A carboxylase 2 (beta) , methylcrotonoyl-CoA carboxylase 2 (beta) , methylcrotonoyl-CoA carboxylase beta chain, mitochondrial-like , 3-methylcrotonyl-CoA carboxylase 2 , 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit , 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta , MCCase subunit beta , biotin carboxylase , non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase

GENE ID SPECIES
405863 Danio rerio
427395 Gallus gallus
461823 Pan troglodytes
478091 Canis lupus familiaris
595048 Xenopus (Silurana) tropicalis
701211 Macaca mulatta
100037195 Xenopus laevis
100414949 Callithrix jacchus
64087 Homo sapiens
361884 Rattus norvegicus
829549 Arabidopsis thaliana
78038 Mus musculus
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