catalog number :
MBS241600
products full name :
Rabbit Polyclonal (IgG) to Human MCAD / ACADM
products short name :
MCAD / ACADM
products name syn :
Anti-MCAD / ACADM Antibody IHC-plus; ACADM; ACAD1; MCAD; MCADH; Human MCAD; ACADM
other names :
medium-chain specific acyl-CoA dehydrogenase, mitochondrial isoform a; Medium-chain specific acyl-CoA dehydrogenase, mitochondrial; medium-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain; acyl-CoA dehydrogenase, C-4 to C-12 straight chain
products gene name :
MCAD
products gene name syn :
ACADM
other gene names :
ACADM; ACADM; MCAD; ACAD1; MCADH; MCAD
uniprot entry name :
ACADM_HUMAN
reactivity :
Human, Mouse, Pig, Sheep
specificity :
Human recombinant MCAD1
purity :
Protein A Purified
form :
Tris buffered saline, 0.1% BSA, 0.02% sodium azide.
concentration :
0.6 mg/ml
storage stability :
Long term: -20 degree C; Short term: +4 degree C. Avoid repeat freeze-thaw cycles.
tested application :
Immunohistochemistry (IHC - Paraffin), Western Blot (WB)
app notes :
IHC-P (10 ug/ml), WB. Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti.
other info1 :
Target Species: Human. Immunogen Description: Recombinant human ACADM.
other info2 :
Immunogen Type: Recombinant protein. Immunogen: MCAD / ACADM antibody was raised against recombinant human ACADM.
products description :
ACADM 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. Clinical phenotypes are associated with ACADM hereditary deficiency.
ncbi acc num :
NP_000007.1
ncbi gb acc num :
NM_000016.5
ncbi mol weight :
47,020 Da
ncbi pathways :
Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway (106127); Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway (106128); Carbon Metabolism Pathway (814926); Carbon Metabolism Pathway (817567); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Degradation Pathway (82935); Fatty Acid Degradation Pathway (296); Fatty Acid Metabolism Pathway (868084); Fatty Acid Metabolism Pathway (878045)
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 summary :
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. Protein type: Carbohydrate Metabolism - propanoate; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Other Amino Acids Metabolism - beta-alanine; Lipid Metabolism - fatty acid; Mitochondrial; EC 1.3.8.7. 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