PCCB Antibody, FITC conjugated (PACO57914)
- SKU:
- PACO57914
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Frequently bought together:
Description
抗体名: | PCCB Antibody, FITC conjugated (PACO57914) |
抗体コード: | PACO57914 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human Propionyl-CoA carboxylase β chain, mitochondrial protein (200-314AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | FITC |
バックグラウンド: | cytosol, mitochondrial matrix, mitochondrion, CoA carboxylase activity, propionyl-CoA carboxylase activity, biotin metabolic process, fatty acid, catabolic process, short-chain fatty acid, catabolic process |
シノニム: | Propionyl-CoA carboxylase β chain, mitochondrial (PCCase subunit beta) (EC 6.4.1.3) (Propanoyl-CoA:carbon dioxide ligase subunit beta), PCCB |
UniProt Protein Function: | PCCB: Defects in PCCB are the cause of propionic acidemia type II (PA-2). PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:EC 6.4.1.3; Ligase; Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial Chromosomal Location of Human Ortholog: 3q21-q22 Cellular Component: mitochondrion; mitochondrial matrix; cytosol Molecular Function:propionyl-CoA carboxylase activity; ATP binding Biological Process: vitamin metabolic process; fatty acid beta-oxidation; short-chain fatty acid catabolic process; cellular lipid metabolic process; biotin metabolic process; water-soluble vitamin metabolic process Disease: Propionic Acidemia |
NCBI Summary: | 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.[provided by RefSeq, May 2010] |
UniProt Code: | P05166 |
NCBI GenInfo Identifier: | 124106304 |
NCBI Gene ID: | 5096 |
NCBI Accession: | P05166.3 |
UniProt Secondary Accession: | P05166,Q16813, Q96CX0, B7Z2Z4, |
UniProt Related Accession: | P05166 |
Molecular Weight: | 60,521 Da |
NCBI Full Name: | Propionyl-CoA carboxylase beta chain, mitochondrial |
NCBI Synonym Full Names: | propionyl CoA carboxylase, beta polypeptide |
NCBI Official Symbol: | PCCB |
NCBI Protein Information: | propionyl-CoA carboxylase beta chain, mitochondrial; PCCase subunit beta; propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl Coenzyme A carboxylase, beta polypeptide |
UniProt Protein Name: | Propionyl-CoA carboxylase beta chain, mitochondrial |
UniProt Synonym Protein Names: | Propanoyl-CoA:carbon dioxide ligase subunit beta |
Protein Family: | Phycoerythrocyanin |
UniProt Gene Name: | PCCB |
UniProt Entry Name: | PCCB_HUMAN |