ACADS Antibody (PACO42933)
- SKU:
- PACO42933
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
抗体名: | ACADS Antibody (PACO42933) |
抗体コード: | PACO42933 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:1000, IHC:1:20-1:200 |
反応性: | Human |
免疫原: | Recombinant Human Short-chain specific acyl-CoA dehydrogenase, mitochondrial protein (1-260AA) |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot. All lanes: Short-chain specific acyl-CoA dehydrogenase, mitochondrial antibody at 7µg/ml. Lane 1: 293T whole cell lysate. Lane 2: HepG2 whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 44 kDa. Observed band size: 44 kDa. |
![]() | Immunohistochemistry of paraffin-embedded human pancreatic tissue using PACO42933 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human liver tissue using PACO42933 at dilution of 1:100. |
シノニム: | Short-chain specific acyl-CoA dehydrogenase, mitochondrial (SCAD) (EC 1.3.8.1) (Butyryl-CoA dehydrogenase), ACADS |
UniProt Protein Function: | ACADS: Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD). It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. Belongs to the acyl-CoA dehydrogenase family. |
UniProt Protein Details: | Protein type:EC 1.3.8.1; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Carbohydrate Metabolism - butanoate; Lipid Metabolism - fatty acid Chromosomal Location of Human Ortholog: 12q24.31 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial membrane; nucleus Molecular Function:acyl-CoA dehydrogenase activity; butyryl-CoA dehydrogenase activity; FAD binding; acyl-CoA binding Biological Process: response to starvation; fatty acid beta-oxidation; response to glucocorticoid stimulus; cellular lipid metabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; butyrate catabolic process; protein homotetramerization Disease: Acyl-coa Dehydrogenase, Short-chain, Deficiency Of |
NCBI Summary: | This gene encodes a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with short-chain acyl-CoA dehydrogenase (SCAD) deficiency. Alternative splicing results in two variants which encode different isoforms. [provided by RefSeq, Oct 2014] |
UniProt Code: | P16219 |
NCBI GenInfo Identifier: | 113019 |
NCBI Gene ID: | 35 |
NCBI Accession: | P16219.1 |
UniProt Secondary Accession: | P16219,P78331, |
UniProt Related Accession: | P16219 |
Molecular Weight: | 412 |
NCBI Full Name: | Short-chain specific acyl-CoA dehydrogenase, mitochondrial |
NCBI Synonym Full Names: | acyl-CoA dehydrogenase, C-2 to C-3 short chain |
NCBI Official Symbol: | ACADS |
NCBI Official Synonym Symbols: | SCAD; ACAD3 |
NCBI Protein Information: | short-chain specific acyl-CoA dehydrogenase, mitochondrial; butyryl-CoA dehydrogenase; unsaturated acyl-CoA reductase; acyl-Coenzyme A dehydrogenase, C-2 to C-3 short chain; mitochondrial short-chain specific acyl-CoA dehydrogenase |
UniProt Protein Name: | Short-chain specific acyl-CoA dehydrogenase, mitochondrial |
UniProt Synonym Protein Names: | Butyryl-CoA dehydrogenase |
Protein Family: | Short-chain specific acyl-CoA dehydrogenase |
UniProt Gene Name: | ACADS |
UniProt Entry Name: | ACADS_HUMAN |