ACADM Antibody, FITC conjugated (PACO24710)
- SKU:
- PACO24710
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- FITC
Description
抗体名: | ACADM Antibody, FITC conjugated (PACO24710) |
抗体コード: | PACO24710 |
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 Medium-chain specific acyl-CoA dehydrogenase, mitochondrial protein (26-421AA) |
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: | Acyl-CoA dehydrogenase specific for acyl chain lengths of 4 to 16 that catalyzes the initial step of fatty acid, beta-oxidation. Utilizes the electron transfer flavoprotein (ETF) as an electron acceptor to transfer electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). |
シノニム: | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial (MCAD) (EC 1.3.8.7), ACADM |
UniProt Protein Function: | ACADM: This enzyme is specific for acyl chain lengths of 4 to 16. Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD). It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:EC 1.3.8.7; Mitochondrial; Carbohydrate Metabolism - propanoate; Oxidoreductase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Other Amino Acids Metabolism - beta-alanine Chromosomal Location of Human Ortholog: 1p31 Cellular Component: mitochondrion; axon; mitochondrial matrix; nucleus Molecular Function:acyl-CoA dehydrogenase activity; identical protein binding; FAD binding Biological Process: carnitine metabolic process, CoA-linked; fatty acid beta-oxidation; medium-chain fatty acid catabolic process; cellular lipid metabolic process; medium-chain fatty acid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; carnitine biosynthetic process Disease: Acyl-coa Dehydrogenase, Medium-chain, Deficiency Of |
NCBI Summary: | This gene encodes the medium-chain specific (C4 to C12 straight chain) acyl-Coenzyme A dehydrogenase. The homotetramer enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Defects in this gene cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | P11310 |
NCBI GenInfo Identifier: | 113017 |
NCBI Gene ID: | 34 |
NCBI Accession: | P11310.1 |
UniProt Related Accession: | P11310 |
Molecular Weight: | |
NCBI Full Name: | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
NCBI Synonym Full Names: | acyl-CoA dehydrogenase medium chain |
NCBI Official Symbol: | ACADM |
NCBI Official Synonym Symbols: | MCAD; ACAD1; MCADH |
NCBI Protein Information: | medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
UniProt Protein Name: | Medium-chain specific acyl-CoA dehydrogenase, mitochondrial |
UniProt Gene Name: | ACADM |
UniProt Entry Name: | ACADM_HUMAN |