ACADM Rabbit Monoclonal Antibody (CAB4567)

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CAB4567
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Description

Technical Manual

ACADM Rabbit Monoclonal Antibody

Product Name:ACADM Rabbit Monoclonal Antibody
Product SKU:CAB4567
Size:20uL, 50 uL
Host Species:Rabbit
Purification Method:Affinity purification
Isotype:IgG
Background:

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]

Immunogen:A synthesized peptide derived from human ACADM
Sequence:

Email for sequence

Gene ID:34
Swiss Prot:P11310
Synonyms:ACAD1; MCAD; MCADH
Calculated MW:46kDa
Observed MW:45KDa
Reactivity:Human, Mouse, Rat
Application:WB IHC IF
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Application Key:Western blotting Immunohistochemistry Immunofluorescence 
Positive Samples:HeLa, 293T, HepG2, Mouse liver, Mouse heart, Rat liver, Rat heart
Cellular Location:

Product Images

ACADM Rabbit Monoclonal Antibody (CAB4567)

Western blot - ACADM Rabbit mAb (CAB4567)

Uniprot Information

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
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Additional Information

Product type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
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