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ACADVL Recombinant monoclonal antibody, PBS Only

ACADVL Uni-rAb® Recombinant Antibody for WB, IF/ICC, Indirect ELISA

Cat No. 85889-1-PBS
Clone No.250199C12

Host / Isotype

Rabbit / IgG

Reactivity

human, mouse

Applications

WB, IF/ICC, Indirect ELISA

VLCAD, EC:1.3.8.9, Very long-chain specific acyl-CoA dehydrogenase, mitochondrial

Formulation:  PBS Only
Conjugate:  Unconjugated
Size/Concentration: 

-/ -

Freight/Packing: -

Quantity

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Product Information

85889-1-PBS targets ACADVL in WB, IF/ICC, Indirect ELISA applications and shows reactivity with human, mouse samples.

Tested Reactivity human, mouse
Host / Isotype Rabbit / IgG
Class Recombinant
Type Antibody
Immunogen

CatNo: Ag6016

Product name: Recombinant human ACADVL protein

Source: e coli.-derived, T-HIS

Tag: 6*His

Domain: 312-655 aa of BC012912

Sequence: FDGVRVPSENVLGEVGSGFKVAMHILNNGRFGMAAALAGTMRGIIAKAVDHATNRTQFGEKIHNFGLIQEKLARMVMLQYVTESMAYMVSANMDQGATDFQIEAAISKIFGSEAAWKVTDECIQIMGGMGFMKEPGVERVLRDLRIFRIFEGTNDILRLFVALQGCMDKGKELSGLGSALKNPFGNAGLLLGEAGKQLRRRAGLGSGLSLSGLVHPELSRSGELAVRALEQFATVVEAKLIKHKKGIVNEQFLLQRLADGAIDLYAMVVVLSRASRSLSEGHPTAQHEKMLCDTWCIEAAARIREGMAALQSDPWQQELYRNFKSISKALVERGGVVTSNPLGF

Predict reactive species
Full Name acyl-Coenzyme A dehydrogenase, very long chain
Calculated Molecular Weight 70 kDa
Observed Molecular Weight60 kDa
GenBank Accession NumberBC012912
Gene Symbol ACADVL
Gene ID (NCBI) 37
Conjugate Unconjugated
FormLiquid
Purification MethodProtein A purification
UNIPROT IDP49748
Storage Buffer PBS only, pH 7.3.
Storage ConditionsStore at -80°C.

Background Information

ACADVL (Acyl-CoA Dehydrogenase, Very Long Chain) is a mitochondrial enzyme that catalyzes the initial step of β-oxidation for very long-chain fatty acids (VLCFAs, C14-C20). Deficiency of ACADVL causes very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common inherited disorder of mitochondrial fatty acid oxidation, characterized by hypoketotic hypoglycemia, cardiomyopathy, hepatopathy, and exercise-induced rhabdomyolysis. ACADVL is highly expressed in metabolically active tissues including cardiac muscle, skeletal muscle, and liver, where it plays a critical role in energy production during fasting and high-demand states. Mutations in ACADVL represent a significant diagnostic target for newborn screening and genetic counseling in metabolic disorders.

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