Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB)

Cat.No	ACP00241	Target Name	PCCB
Target Synonyms	PCCase subunit beta;Propanoyl-CoA:carbon dioxide ligase subunit beta	Form	Liquid or Lyophilized powder
Expression System	E.coli	Expression Range	29-539aa
Mol Weight	62.4 kDa	Protein Length	Full Length of Mature Protein
Purity	Greater than 90% as determined by SDS-PAGE.	Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Cat.No

ACP00241

Target Name

PCCB

Target Synonyms

PCCase subunit beta;Propanoyl-CoA:carbon dioxide ligase subunit beta

Form

Liquid or Lyophilized powder

Expression System

E.coli

Expression Range

29-539aa

Mol Weight

62.4 kDa

Protein Length

Full Length of Mature Protein

Purity

Greater than 90% as determined by SDS-PAGE.

Storage Buffer

5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Target Species	Human	Uniprot ID	P05166

Background Information

Uniprot Id

P05166
Target Species

Human
Target Name

PCCB
Target Full Name

Propionyl-CoA carboxylase beta chain, mitochondrial
Target Function

This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites. Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA. Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA. Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate. Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA.
Target Involvement

Propionic acidemia type II (PA-2)
Target Subcellular Location

Mitochondrion matrix.
Target Protein Families

AccD/PCCB family
Target Research Area

Metabolism
Target Synonyms

DKFZp451E113; PCCase subunit beta; pccB; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carboxylase beta chain; mitochondrial ; propionyl Coenzyme A carboxylase; beta polypeptide; Propionyl-CoA carboxylase ; beta subunit ; Propionyl-CoA carboxylase beta chain; mitochondrial; R74805
Target Background

The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.

Recombinant Human Propionyl-CoA carboxylase beta chain, mitochondrial (PCCB)

Specifications

Immunogen Information

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