Name: ACADV Polyclonal Antibody
SKU: WP2852
Price: 268 USD
Availability: InStock

ACADV Polyclonal Antibody

Ucallm_WP2852-50

Price range: $268.00 through $328.00

Size

100μl

50μl

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SKU: WP2852-100

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Product details

Background:

acyl-CoA dehydrogenase, very long chain(ACADVL) Homo sapiens The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008],

Specifications

Size	50μL/100μL
Target	ACADV
Reactivity	Human, Mouse
Applications	WB, ELISA
MW(Calculated)
MW(Observed)	72kD
Host Species	Rabbit
Isotype	IgG
Conjugate/Modification	Unmodified
Recommended Dilution Ratio	WB 1:500-2000; ELISA 1:5000-20000
Formulation	Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide.
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration	1 mg/ml
Clonality	Polyclonal
Clone Number
Immunogen	Synthesized peptide derived from human protein . at AA range: 471-520
Specificity	ACADV Polyclonal Antibody detects endogenous levels of protein.
Gene Name	ACADVL VLCAD
Protein Name	Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) (EC 1.3.8.9)
Other Name
Species	Human
Gene ID	37
Uniprot	P49748,
Species.1	Mouse
Gene ID.1
Uniprot.1	P50544,
Species.2	Rat
Gene ID.2
Uniprot.2	P45953
Cellular Localization	Mitochondrion inner membrane; Peripheral membrane protein .; [Isoform 2]: Mitochondrion inner membrane; Peripheral membrane protein .
Tissue Expression	Adipose tissue,Liver,Lung,Pancreas,Peripheral blood,Placenta,
Signaling_pathway
Research Areas	>>Fatty acid degradation; / >>Metabolic pathways; / >>Fatty acid metabolism; / >>Alcoholic liver disease
Function	Catalytic activity:Acyl-CoA + ETF = 2,3-dehydroacyl-CoA + reduced ETF.,cofactor:FAD.,Disease:Defects in ACADVL are the cause of very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) [MIM:201475]. VLCAD deficiency is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting.,Function:Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.,miscellaneous:A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.,pathway:Lipid metabolism; mitochondrial fatty acid beta-oxidation.,similarity:Belongs to the acyl-CoA dehydrogenase family.,subunit:Homodimer.,

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ACADV Polyclonal Antibody

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ACADV Polyclonal Antibody

Product details

Specifications

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