ACADM Antibody (PACO43407)
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주문- SKU:
- PACO43407
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
Antibody Name: | ACADM Antibody (PACO43407) |
Antibody SKU: | PACO43407 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Medium-chain specific acyl-CoA dehydrogenase, mitochondrial protein (26-421AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Immunohistochemistry of paraffin-embedded human kidney tissue using PACO43407 at dilution of 1:100. |
Background: | This enzyme is specific for acyl chain lengths of 4 to 16. |
Synonyms: | 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 |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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