Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA)

Cat.No	ACP00243	Target Name	PCCA
Target Synonyms	PCCase subunit alpha;Propanoyl-CoA:carbon dioxide ligase subunit alpha	Form	Liquid or Lyophilized powder
Expression System	E.coli	Expression Range	53-728aa
Mol Weight	81.2 kDa	Protein Length	Full Length of Mature Protein
Purity	Greater than 95% 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

ACP00243

Target Name

PCCA

Target Synonyms

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

Form

Liquid or Lyophilized powder

Expression System

E.coli

Expression Range

53-728aa

Mol Weight

81.2 kDa

Protein Length

Full Length of Mature Protein

Purity

Greater than 95% 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	P05165

Background Information

Uniprot Id

P05165
Target Species

Human
Target Name

PCCA
Target Full Name

Propionyl-CoA carboxylase alpha 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 I (PA-1)
Target Subcellular Location

Mitochondrion matrix.
Target Research Area

Metabolism
Target Synonyms

mitochondrial; Pcca; pccA COMPLEMENTATION GROUP; PCCA_HUMAN; PCCase subunit alpha; Propanoyl CoA:carbon dioxide ligase subunit alpha ; Propanoyl-CoA:carbon dioxide ligase subunit alpha; Propionyl CoA carboxylase alpha chain; Propionyl CoA carboxylase alpha chain mitochondrial; Propionyl CoA carboxylase alpha polypeptide; Propionyl coenzyme A carboxylase alpha polypeptide ; Propionyl-CoA carboxylase alpha chain
Target Background

The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.

Recombinant Human Propionyl-CoA carboxylase alpha chain, mitochondrial (PCCA)

Specifications

Immunogen Information

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