ACADL Antibody, HRP conjugated (PACO24705)
- SKU:
- PACO24705
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Description
抗体名: | ACADL Antibody, HRP conjugated (PACO24705) |
抗体コード: | PACO24705 |
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 Long-chain specific acyl-CoA dehydrogenase, mitochondrial protein (30-260AA) |
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: | HRP |
バックグラウンド: | mitochondrial matrix, acyl-CoA dehydrogenase activity, electron carrier activity, fatty-acyl-CoA binding, flavin adenine dinucleotide binding, long-chain-acyl-CoA dehydrogenase activity, oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor, palmitoyl-CoA oxidase activity, carnitine catabolic process, carnitine metabolic process, CoA-linked |
シノニム: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial (LCAD) (EC 1.3.8.8), ACADL |
UniProt Protein Function: | ACADL: Defects in ACADL are a cause of acyl-CoA dehydrogenase very long-chain deficiency (ACADVLD). An inborn error of mitochondrial fatty acid beta-oxidation which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form characterized by early onset, high mortality and high incidence of cardiomyopathy; a milder childhood form with later onset, characterized by hypoketotic hypoglycemia, low mortality and rare cardiomyopathy; an adult form, with isolated skeletal muscle involvement, rhabdomyolysis and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. |
UniProt Protein Details: | Protein type:Mitochondrial; Lipid Metabolism - fatty acid; EC 1.3.8.8; Oxidoreductase Chromosomal Location of Human Ortholog: 2q34 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; cytoplasm Molecular Function:palmitoyl-CoA oxidase activity; acyl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding; long-chain-acyl-CoA dehydrogenase activity Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; cellular lipid catabolic process; carnitine catabolic process; thermoregulation; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid oxidation; negative regulation of fatty acid biosynthetic process; long-chain fatty acid catabolic process; protein homotetramerization |
NCBI Summary: | The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008] |
UniProt Code: | P28330 |
NCBI GenInfo Identifier: | 223590148 |
NCBI Gene ID: | 33 |
NCBI Accession: | P28330.2 |
UniProt Related Accession: | P28330 |
Molecular Weight: | |
NCBI Full Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
NCBI Synonym Full Names: | acyl-CoA dehydrogenase long chain |
NCBI Official Symbol: | ACADL |
NCBI Official Synonym Symbols: | LCAD; ACAD4 |
NCBI Protein Information: | long-chain specific acyl-CoA dehydrogenase, mitochondrial |
UniProt Protein Name: | Long-chain specific acyl-CoA dehydrogenase, mitochondrial |
Protein Family: | Long-chain specific acyl-CoA dehydrogenase |
UniProt Gene Name: | ACADL |
UniProt Entry Name: | ACADL_HUMAN |